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Entry version 174 (18 Sep 2019)
Sequence version 2 (31 Jan 2002)
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Protein

NLR family CARD domain-containing protein 4

Gene

NLRC4

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Key component of inflammasomes that indirectly senses specific proteins from pathogenic bacteria and fungi and responds by assembling an inflammasome complex that promotes caspase-1 activation, cytokine production and macrophage pyroptosis (PubMed:15107016). The NLRC4 inflammasome is activated as part of the innate immune response to a range of intracellular bacteria (By similarity).By similarity1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi169 – 176ATPPROSITE-ProRule annotation8

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Biological processApoptosis, Immunity, Inflammatory response, Innate immunity
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-6803207 TP53 Regulates Transcription of Caspase Activators and Caspases
R-HSA-844623 The IPAF inflammasome

SIGNOR Signaling Network Open Resource

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SIGNORi
Q9NPP4

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
NLR family CARD domain-containing protein 4
Alternative name(s):
CARD, LRR, and NACHT-containing protein
Short name:
Clan protein
Caspase recruitment domain-containing protein 12
Ice protease-activating factor
Short name:
Ipaf
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:NLRC4
Synonyms:CARD12, CLAN, CLAN1, IPAF
ORF Names:UNQ6189/PRO20215
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 2

Organism-specific databases

Human Gene Nomenclature Database

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HGNCi
HGNC:16412 NLRC4

Online Mendelian Inheritance in Man (OMIM)

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MIMi
606831 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q9NPP4

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Autoinflammation with infantile enterocolitis (AIFEC)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal dominant disorder characterized by neonatal-onset enterocolitis, periodic fever, and fatal or near-fatal episodes of autoinflammation. Affected individuals tend to have poor overall growth and gastrointestinal symptoms in infancy, recurrent febrile episodes with splenomegaly, and sometimes hematologic disturbances, arthralgias, or myalgias.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_072484337T → S in AIFEC; results in a gain of function mutation with constitutive activation of caspase-1. 1 PublicationCorresponds to variant dbSNP:rs587777840EnsemblClinVar.1
Natural variantiVAR_072485341V → A in AIFEC; results in a gain of function mutation with constitutive activation of caspase-1. 1 PublicationCorresponds to variant dbSNP:rs587781260EnsemblClinVar.1
Familial cold autoinflammatory syndrome 4 (FCAS4)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of autoinflammatory syndrome, a rare autosomal dominant systemic disease characterized by recurrent episodes of maculopapular rash associated with arthralgias, myalgias, fever and chills, swelling of the extremities, and conjunctivitis after generalized exposure to cold.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_072645443H → P in FCAS4; the mutation increases oligomerization of the NLRC4 protein; results in hyperactivation of caspase-1 with an increase in IL1B protein secretion. 1 PublicationCorresponds to variant dbSNP:rs606231460EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNET

More...
DisGeNETi
58484

MalaCards human disease database

More...
MalaCardsi
NLRC4
MIMi616050 phenotype
616115 phenotype

Open Targets

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OpenTargetsi
ENSG00000091106

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
1451 CINCA syndrome
47045 Familial cold urticaria
436166 Periodic fever-infantile enterocolitis-autoinflammatory syndrome

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA162397671

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
NLRC4

Domain mapping of disease mutations (DMDM)

More...
DMDMi
20138032

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001440871 – 1024NLR family CARD domain-containing protein 4Add BLAST1024

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei533PhosphoserineBy similarity1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Phosphorylated at Ser-533 following infection of macrophages with S.typhimurium (Salmonella). Phosphorylation is essential for NLRC4 inflammasome function to promote caspase-1 activation and pyroptosis. PRKCD phosphorylates Ser-533 in vitro.By similarity

Keywords - PTMi

Phosphoprotein

Proteomic databases

jPOST - Japan Proteome Standard Repository/Database

More...
jPOSTi
Q9NPP4

MassIVE - Mass Spectrometry Interactive Virtual Environment

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MassIVEi
Q9NPP4

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
Q9NPP4

PeptideAtlas

More...
PeptideAtlasi
Q9NPP4

PRoteomics IDEntifications database

More...
PRIDEi
Q9NPP4

ProteomicsDB human proteome resource

More...
ProteomicsDBi
82036 [Q9NPP4-1]
82037 [Q9NPP4-2]
82038 [Q9NPP4-3]
82039 [Q9NPP4-4]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
Q9NPP4

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...
PhosphoSitePlusi
Q9NPP4

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Isoform 2 is expressed ubiquitously, although highly expressed in lung and spleen. Isoform 1 is highly expressed in lung, followed by leukocytes especially monocytes, lymph node, colon, brain, prostate, placenta, spleen, bone marrow and fetal liver. Isoform 4 is only detected in brain.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000091106 Expressed in 106 organ(s), highest expression level in bone marrow

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
Q9NPP4 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA006592

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homooligomer (PubMed:25385754). Homooligomerizes following activation of Naip proteins by pathogenic proteins such as S.typhimurium (Salmonella) flagellin or PrgJ.

Component of the NLRC4 inflammasome, at least composed of NLRC4, caspase-1 (CASP1) and some NAIP family member (By similarity).

Interacts with ASC, pro-caspase-1, NOD2, BCL10 and NALP1 (NAC) by CARD-CARD interaction.

Interacts with EIF2AK2/PKR (PubMed:22801494).

By similarity1 Publication

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
121814, 14 interactors

Database of interacting proteins

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DIPi
DIP-38428N

Protein interaction database and analysis system

More...
IntActi
Q9NPP4, 8 interactors

STRING: functional protein association networks

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STRINGi
9606.ENSP00000385090

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

11024
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
Q9NPP4

Database of comparative protein structure models

More...
ModBasei
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini1 – 88CARDPROSITE-ProRule annotationAdd BLAST88
Domaini163 – 476NACHTPROSITE-ProRule annotationAdd BLAST314
<p>This subsection of the ‘Family and Domains’ section indicates the positions and types of repeated sequence motifs or repeated domains within the protein.<p><a href='/help/repeat' target='_top'>More...</a></p>Repeati578 – 598LRR 1Add BLAST21
Repeati656 – 679LRR 2Add BLAST24
Repeati735 – 758LRR 3Add BLAST24
Repeati762 – 785LRR 4Add BLAST24
Repeati787 – 812LRR 5Add BLAST26
Repeati824 – 847LRR 6Add BLAST24
Repeati848 – 870LRR 7Add BLAST23
Repeati878 – 902LRR 8Add BLAST25
Repeati911 – 933LRR 9Add BLAST23
Repeati936 – 963LRR 10Add BLAST28
Repeati965 – 985LRR 11Add BLAST21
Repeati999 – 1021LRR 12Add BLAST23

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni95 – 298Nucleotide-binding domain (NBD)By similarityAdd BLAST204
Regioni356 – 463Winged-helix domain (WHD)By similarityAdd BLAST108

<p>This subsection of the ‘Family and domains’ section provides general information on the biological role of a domain. The term ‘domain’ is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

In an autoinhibited form the C-terminal leucine-rich repeat (LRR) domain is positioned to sterically occlude one side of the NBD domain and consequently sequester NLRC4 in a monomeric state. An ADP-mediated interaction between the NBD and the WHD also contributes to the autoinhibition.By similarity

Keywords - Domaini

Leucine-rich repeat, Repeat

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
ENOG410IHIG Eukaryota
ENOG410YI2E LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000161744

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000059630

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q9NPP4

KEGG Orthology (KO)

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KOi
K12805

Identification of Orthologs from Complete Genome Data

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OMAi
MASWEKA

Database of Orthologous Groups

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OrthoDBi
137566at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
Q9NPP4

TreeFam database of animal gene trees

More...
TreeFami
TF336864

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
3.80.10.10, 2 hits

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR001315 CARD
IPR011029 DEATH-like_dom_sf
IPR032675 LRR_dom_sf
IPR007111 NACHT_NTPase
IPR042220 NLRC4
IPR040535 NLRC4_HD
IPR027417 P-loop_NTPase

The PANTHER Classification System

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PANTHERi
PTHR47688 PTHR47688, 1 hit

Pfam protein domain database

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Pfami
View protein in Pfam
PF00619 CARD, 1 hit
PF05729 NACHT, 1 hit
PF17889 NLRC4_HD, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF47986 SSF47986, 1 hit
SSF52540 SSF52540, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS50209 CARD, 1 hit
PS50837 NACHT, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (4+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

This entry describes 4 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 4 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9NPP4-1) [UniParc]FASTAAdd to basket
Also known as: CLANA

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MNFIKDNSRA LIQRMGMTVI KQITDDLFVW NVLNREEVNI ICCEKVEQDA
60 70 80 90 100
ARGIIHMILK KGSESCNLFL KSLKEWNYPL FQDLNGQSLF HQTSEGDLDD
110 120 130 140 150
LAQDLKDLYH TPSFLNFYPL GEDIDIIFNL KSTFTEPVLW RKDQHHHRVE
160 170 180 190 200
QLTLNGLLQA LQSPCIIEGE SGKGKSTLLQ RIAMLWGSGK CKALTKFKFV
210 220 230 240 250
FFLRLSRAQG GLFETLCDQL LDIPGTIRKQ TFMAMLLKLR QRVLFLLDGY
260 270 280 290 300
NEFKPQNCPE IEALIKENHR FKNMVIVTTT TECLRHIRQF GALTAEVGDM
310 320 330 340 350
TEDSAQALIR EVLIKELAEG LLLQIQKSRC LRNLMKTPLF VVITCAIQMG
360 370 380 390 400
ESEFHSHTQT TLFHTFYDLL IQKNKHKHKG VAASDFIRSL DHCGDLALEG
410 420 430 440 450
VFSHKFDFEL QDVSSVNEDV LLTTGLLCKY TAQRFKPKYK FFHKSFQEYT
460 470 480 490 500
AGRRLSSLLT SHEPEEVTKG NGYLQKMVSI SDITSTYSSL LRYTCGSSVE
510 520 530 540 550
ATRAVMKHLA AVYQHGCLLG LSIAKRPLWR QESLQSVKNT TEQEILKAIN
560 570 580 590 600
INSFVECGIH LYQESTSKSA LSQEFEAFFQ GKSLYINSGN IPDYLFDFFE
610 620 630 640 650
HLPNCASALD FIKLDFYGGA MASWEKAAED TGGIHMEEAP ETYIPSRAVS
660 670 680 690 700
LFFNWKQEFR TLEVTLRDFS KLNKQDIRYL GKIFSSATSL RLQIKRCAGV
710 720 730 740 750
AGSLSLVLST CKNIYSLMVE ASPLTIEDER HITSVTNLKT LSIHDLQNQR
760 770 780 790 800
LPGGLTDSLG NLKNLTKLIM DNIKMNEEDA IKLAEGLKNL KKMCLFHLTH
810 820 830 840 850
LSDIGEGMDY IVKSLSSEPC DLEEIQLVSC CLSANAVKIL AQNLHNLVKL
860 870 880 890 900
SILDLSENYL EKDGNEALHE LIDRMNVLEQ LTALMLPWGC DVQGSLSSLL
910 920 930 940 950
KHLEEVPQLV KLGLKNWRLT DTEIRILGAF FGKNPLKNFQ QLNLAGNRVS
960 970 980 990 1000
SDGWLAFMGV FENLKQLVFF DFSTKEFLPD PALVRKLSQV LSKLTFLQEA
1010 1020
RLVGWQFDDD DLSVITGAFK LVTA
Length:1,024
Mass (Da):116,159
Last modified:January 31, 2002 - v2
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i49378DBB54938E0F
GO
Isoform 2 (identifier: Q9NPP4-2) [UniParc]FASTAAdd to basket
Also known as: CLANB

The sequence of this isoform differs from the canonical sequence as follows:
     89-753: Missing.

Show »
Length:359
Mass (Da):40,623
Checksum:i764417DD658B1DFD
GO
Isoform 3 (identifier: Q9NPP4-3) [UniParc]FASTAAdd to basket
Also known as: CLANC

The sequence of this isoform differs from the canonical sequence as follows:
     155-156: NG → VL
     157-1024: Missing.

Show »
Length:156
Mass (Da):18,295
Checksum:i933CB2315528D67A
GO
Isoform 4 (identifier: Q9NPP4-4) [UniParc]FASTAAdd to basket
Also known as: CLAND

The sequence of this isoform differs from the canonical sequence as follows:
     90-92: FHQ → LTA
     93-1024: Missing.

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Length:92
Mass (Da):10,646
Checksum:iBD090CD064330D1D
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A494BZZ1A0A494BZZ1_HUMAN
NLR family CARD domain-containing p...
NLRC4
764Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A499FIV7A0A499FIV7_HUMAN
NLR family CARD domain-containing p...
NLRC4
794Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti39N → D in BAF85362 (PubMed:14702039).Curated1
Sequence conflicti138V → I in AAK14776 (PubMed:11472070).Curated1
Sequence conflicti393C → R in AAK14776 (PubMed:11472070).Curated1
Sequence conflicti420V → A in AAK53443 (Ref. 4) Curated1
Sequence conflicti678R → T in AAK38730 (PubMed:11374873).Curated1
Sequence conflicti791K → E in BAG53062 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_072484337T → S in AIFEC; results in a gain of function mutation with constitutive activation of caspase-1. 1 PublicationCorresponds to variant dbSNP:rs587777840EnsemblClinVar.1
Natural variantiVAR_072485341V → A in AIFEC; results in a gain of function mutation with constitutive activation of caspase-1. 1 PublicationCorresponds to variant dbSNP:rs587781260EnsemblClinVar.1
Natural variantiVAR_072645443H → P in FCAS4; the mutation increases oligomerization of the NLRC4 protein; results in hyperactivation of caspase-1 with an increase in IL1B protein secretion. 1 PublicationCorresponds to variant dbSNP:rs606231460EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_00078489 – 753Missing in isoform 2. 1 PublicationAdd BLAST665
Alternative sequenceiVSP_00078790 – 92FHQ → LTA in isoform 4. 1 Publication3
Alternative sequenceiVSP_00078893 – 1024Missing in isoform 4. 1 PublicationAdd BLAST932
Alternative sequenceiVSP_000785155 – 156NG → VL in isoform 3. 1 Publication2
Alternative sequenceiVSP_000786157 – 1024Missing in isoform 3. 1 PublicationAdd BLAST868

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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DDBJi
Links Updated
AY032589 mRNA Translation: AAK38730.1
AY027787 mRNA Translation: AAK14776.1
AY027788 mRNA Translation: AAK14777.1
AY027789 mRNA Translation: AAK14778.1
AY027790 mRNA Translation: AAK14779.1
AY035391 mRNA Translation: AAK59843.1
AF376061 mRNA Translation: AAK53443.1
AY358152 mRNA Translation: AAQ88519.1
AK095467 mRNA Translation: BAG53062.1
AK292673 mRNA Translation: BAF85362.1
AK314762 mRNA Translation: BAG37300.1
AL121653 Genomic DNA No translation available.
CH471053 Genomic DNA Translation: EAX00452.1
CH471053 Genomic DNA Translation: EAX00453.1
CH471053 Genomic DNA Translation: EAX00454.1
CH471053 Genomic DNA Translation: EAX00455.1
BC031555 mRNA Translation: AAH31555.1
AL389934 mRNA Translation: CAB97523.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS33174.1 [Q9NPP4-1]
CCDS77400.1 [Q9NPP4-2]

NCBI Reference Sequences

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RefSeqi
NP_001186067.1, NM_001199138.1 [Q9NPP4-1]
NP_001186068.1, NM_001199139.1 [Q9NPP4-1]
NP_001289433.1, NM_001302504.1 [Q9NPP4-2]
NP_067032.3, NM_021209.4 [Q9NPP4-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000342905; ENSP00000339666; ENSG00000091106 [Q9NPP4-2]
ENST00000360906; ENSP00000354159; ENSG00000091106 [Q9NPP4-1]
ENST00000402280; ENSP00000385428; ENSG00000091106 [Q9NPP4-1]

Database of genes from NCBI RefSeq genomes

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GeneIDi
58484

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:58484

UCSC genome browser

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UCSCi
uc002roi.4 human [Q9NPP4-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY032589 mRNA Translation: AAK38730.1
AY027787 mRNA Translation: AAK14776.1
AY027788 mRNA Translation: AAK14777.1
AY027789 mRNA Translation: AAK14778.1
AY027790 mRNA Translation: AAK14779.1
AY035391 mRNA Translation: AAK59843.1
AF376061 mRNA Translation: AAK53443.1
AY358152 mRNA Translation: AAQ88519.1
AK095467 mRNA Translation: BAG53062.1
AK292673 mRNA Translation: BAF85362.1
AK314762 mRNA Translation: BAG37300.1
AL121653 Genomic DNA No translation available.
CH471053 Genomic DNA Translation: EAX00452.1
CH471053 Genomic DNA Translation: EAX00453.1
CH471053 Genomic DNA Translation: EAX00454.1
CH471053 Genomic DNA Translation: EAX00455.1
BC031555 mRNA Translation: AAH31555.1
AL389934 mRNA Translation: CAB97523.1
CCDSiCCDS33174.1 [Q9NPP4-1]
CCDS77400.1 [Q9NPP4-2]
RefSeqiNP_001186067.1, NM_001199138.1 [Q9NPP4-1]
NP_001186068.1, NM_001199139.1 [Q9NPP4-1]
NP_001289433.1, NM_001302504.1 [Q9NPP4-2]
NP_067032.3, NM_021209.4 [Q9NPP4-1]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
6MKSelectron microscopy3.40A/B/C/D/E/F/G/H/I/J/K/L/M/N/O/P/Q/R/S/T/U/V/W/X/Y/Z/a/b/c/d1-96[»]
6N1Ielectron microscopy3.58A/B/C/D/E/F/G/H/I/J/K/L/M/N/O/P1-85[»]
SMRiQ9NPP4
ModBaseiSearch...

Protein-protein interaction databases

BioGridi121814, 14 interactors
DIPiDIP-38428N
IntActiQ9NPP4, 8 interactors
STRINGi9606.ENSP00000385090

PTM databases

iPTMnetiQ9NPP4
PhosphoSitePlusiQ9NPP4

Polymorphism and mutation databases

BioMutaiNLRC4
DMDMi20138032

Proteomic databases

jPOSTiQ9NPP4
MassIVEiQ9NPP4
PaxDbiQ9NPP4
PeptideAtlasiQ9NPP4
PRIDEiQ9NPP4
ProteomicsDBi82036 [Q9NPP4-1]
82037 [Q9NPP4-2]
82038 [Q9NPP4-3]
82039 [Q9NPP4-4]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
58484
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000342905; ENSP00000339666; ENSG00000091106 [Q9NPP4-2]
ENST00000360906; ENSP00000354159; ENSG00000091106 [Q9NPP4-1]
ENST00000402280; ENSP00000385428; ENSG00000091106 [Q9NPP4-1]
GeneIDi58484
KEGGihsa:58484
UCSCiuc002roi.4 human [Q9NPP4-1]

Organism-specific databases

Comparative Toxicogenomics Database

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CTDi
58484
DisGeNETi58484

GeneCards: human genes, protein and diseases

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GeneCardsi
NLRC4
HGNCiHGNC:16412 NLRC4
HPAiHPA006592
MalaCardsiNLRC4
MIMi606831 gene
616050 phenotype
616115 phenotype
neXtProtiNX_Q9NPP4
OpenTargetsiENSG00000091106
Orphaneti1451 CINCA syndrome
47045 Familial cold urticaria
436166 Periodic fever-infantile enterocolitis-autoinflammatory syndrome
PharmGKBiPA162397671

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiENOG410IHIG Eukaryota
ENOG410YI2E LUCA
GeneTreeiENSGT00940000161744
HOGENOMiHOG000059630
InParanoidiQ9NPP4
KOiK12805
OMAiMASWEKA
OrthoDBi137566at2759
PhylomeDBiQ9NPP4
TreeFamiTF336864

Enzyme and pathway databases

ReactomeiR-HSA-6803207 TP53 Regulates Transcription of Caspase Activators and Caspases
R-HSA-844623 The IPAF inflammasome
SIGNORiQ9NPP4

Miscellaneous databases

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
NLRC4

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
58484

Pharos

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Pharosi
Q9NPP4

Protein Ontology

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PROi
PR:Q9NPP4

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

Gene expression databases

BgeeiENSG00000091106 Expressed in 106 organ(s), highest expression level in bone marrow
GenevisibleiQ9NPP4 HS

Family and domain databases

Gene3Di3.80.10.10, 2 hits
InterProiView protein in InterPro
IPR001315 CARD
IPR011029 DEATH-like_dom_sf
IPR032675 LRR_dom_sf
IPR007111 NACHT_NTPase
IPR042220 NLRC4
IPR040535 NLRC4_HD
IPR027417 P-loop_NTPase
PANTHERiPTHR47688 PTHR47688, 1 hit
PfamiView protein in Pfam
PF00619 CARD, 1 hit
PF05729 NACHT, 1 hit
PF17889 NLRC4_HD, 1 hit
SUPFAMiSSF47986 SSF47986, 1 hit
SSF52540 SSF52540, 1 hit
PROSITEiView protein in PROSITE
PS50209 CARD, 1 hit
PS50837 NACHT, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiNLRC4_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q9NPP4
Secondary accession number(s): A8K9F8
, B2RBQ3, B3KTF0, D6W580, Q96J81, Q96J82, Q96J83
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 31, 2002
Last sequence update: January 31, 2002
Last modified: September 18, 2019
This is version 174 of the entry and version 2 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  2. Human chromosome 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
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