| http://purl.uniprot.org/citations/26393339 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/26393339 | http://www.w3.org/2000/01/rdf-schema#comment | "Protein zero (P0) is the major structural component of peripheral myelin. Lack of this adhesion protein from Schwann cells causes a severe dysmyelinating neuropathy with secondary axonal degeneration in humans with the neuropathy Dejerine-Sottas syndrome (DSS) and in the corresponding mouse model (P0(null)-mice). In the mammalian CNS, the tetraspan-membrane protein PLP is the major structural myelin constituent and required for the long-term preservation of myelinated axons, which fails in hereditary spastic paraplegia (SPG type-2) and the relevant mouse model (Plp(null)-mice). The Plp-gene is also expressed in Schwann cells but PLP is of very low abundance in normal peripheral myelin; its function has thus remained enigmatic. Here we show that the abundance of PLP but not of other tetraspan myelin proteins is strongly increased in compact peripheral myelin of P0(null)-mice. To determine the functional relevance of PLP expression in the absence of P0, we generated P0(null)*Plp(null)-double-mutant mice. Compared with either single-mutant, P0(null)*Plp(null)-mice display impaired nerve conduction, reduced motor functions, and premature death. At the morphological level, axonal segments were frequently non-myelinated but in a one-to-one relationship with a hypertrophic Schwann cell. Importantly, axonal numbers were reduced in the vital phrenic nerve of P0(null)*Plp(null)-mice. In the absence of P0, thus, PLP also contributes to myelination by Schwann cells and to the preservation of peripheral axons. These data provide a link between the Schwann cell-dependent support of peripheral axons and the oligodendrocyte-dependent support of central axons."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.org/dc/terms/identifier | "doi:10.1002/glia.22922"xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Nave K.A."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Werner H.B."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Martini R."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Sereda M.W."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Mobius W."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Patzig J."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Fledrich R."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Kusch K."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Eichel M.A."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/author | "Luders K.A."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/date | "2016"xsd:gYear |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/name | "Glia"xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/pages | "155-174"xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/title | "Proteolipid protein modulates preservation of peripheral axons and premature death when myelin protein zero is lacking."xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://purl.uniprot.org/core/volume | "64"xsd:string |
| http://purl.uniprot.org/citations/26393339 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/26393339 |
| http://purl.uniprot.org/citations/26393339 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/26393339 |
| http://purl.uniprot.org/uniprot/#_A0A5F8MPM4-mappedCitation-26393339 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26393339 |
| http://purl.uniprot.org/uniprot/#_E9QK82-mappedCitation-26393339 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26393339 |
| http://purl.uniprot.org/uniprot/#_Q62079-mappedCitation-26393339 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26393339 |
| http://purl.uniprot.org/uniprot/#_P27573-mappedCitation-26393339 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26393339 |
| http://purl.uniprot.org/uniprot/#_P60202-mappedCitation-26393339 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26393339 |