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http://purl.uniprot.org/citations/28743268http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/28743268http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/28743268http://www.w3.org/2000/01/rdf-schema#comment"Haploinsufficiency of GRN, the gene encoding progranulin (PGRN), causes frontotemporal lobar degeneration (FTLD), the second most common cause of early-onset dementia. Receptor-mediated lysosomal targeting has been shown to regulate brain PGRN levels, and complete deficiency of PGRN is a direct cause of neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disease. Here we show that the lysosomal cysteine protease cathepsin L (Cat L) can mediate the proteolytic cleavage of intracellular PGRN into poly-granulin and granulin fragments. Further, PGRN and Cat L co-localize in lysosomes of HEK293 cells, iPSC-derived neurons and human cortical neurons from human postmortem tissue. These data identify Cat L as a key intracellular lysosomal PGRN protease, and provides an intriguing new link between lysosomal dysfunction and FTLD."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.org/dc/terms/identifier"doi:10.1186/s13024-017-0196-6"xsd:string
http://purl.uniprot.org/citations/28743268http://purl.org/dc/terms/identifier"doi:10.1186/s13024-017-0196-6"xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Lam Y.W."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Lam Y.W."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Bogyo M."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Bogyo M."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Dickson D.W."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Dickson D.W."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Prudencio M."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Prudencio M."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Lau K.F."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Lau K.F."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Lee C.W."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Lee C.W."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Petrucelli L."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Petrucelli L."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Gao F.B."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Gao F.B."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Carlomagno Y."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Carlomagno Y."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Almeida S."xsd:string
http://purl.uniprot.org/citations/28743268http://purl.uniprot.org/core/author"Almeida S."xsd:string