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Details on Person UniProt:Q15149 PLEC

Class:IdReferenceGeneProduct:69847
_chainChangeLogchain:1-4684 added on Fri February 6 2015
_displayNameUniProt:Q15149 PLEC
_timestamp2024-11-03 19:57:59
chainchain:1-4684
checksum04772E4F70A304C8
commentFUNCTION Interlinks intermediate filaments with microtubules and microfilaments and anchors intermediate filaments to desmosomes or hemidesmosomes. Could also bind muscle proteins such as actin to membrane complexes in muscle. May be involved not only in the filaments network, but also in the regulation of their dynamics. Structural component of muscle. Isoform 9 plays a major role in the maintenance of myofiber integrity.SUBUNIT Homodimer or homotetramer. Interacts (via actin-binding domain) with SYNE3. Interacts (via calponin-homology (CH) 1 domain) with VIM (via rod region) (By similarity). Interacts (via N-terminus) with DST isoform 2 (via N-terminus) (PubMed:19932097). Interacts with FER. Interacts with TOR1A (PubMed:18827015). Interacts with ANK3 (PubMed:21223964). Identified in complexes that contain VIM, EZR, AHNAK, BFSP1, BFSP2, ANK2, PLEC, PRX and spectrin (By similarity). Interacts with COL17A1 (PubMed:12482924).SUBUNIT Interacts with KRT14, heterodimers consisting of KRT8 and KRT18, heterodimers consisting of KRT5 and KRT14, heterodimers consisting of KRT14 and KRT15, and heterodimers consisting of KRT1 and KRT10 (PubMed:24940650). Interacts with DES and VIM (PubMed:24940650).INTERACTION Localized to the cortex of myotube podosomes.ALTERNATIVE PRODUCTS Widely expressed with highest levels in muscle, heart, placenta and spinal cord.DOMAIN The N-terminus interacts with actin, the C-terminus with vimentin, desmin, GFAP, cytokeratins, lamin B; whereas both the N- and the C-terminus can bind integrin beta-4.PTM Phosphorylated by CDK1; regulates dissociation from intermediate filaments during mitosis.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry. A 9 bp deletion containing the initiation codon in exon 1f of PLEC have been found in limb-girdle muscular dystrophy patients. The mutation results in deficient expression of isoform 9 and disorganization of the myofibers, without any effect on the skin.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the plakin or cytolinker family.ONLINE INFORMATION Plectin entry
descriptionrecommendedName: Plectin shortName: PCN shortName: PLTN alternativeName: Hemidesmosomal protein 1 shortName: HD1 alternativeName: Plectin-1
geneNamePLEC
PLEC1
identifierQ15149
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Actin-binding
Alternative splicing
Cell junction
Cell projection
Coiled coil
Cytoplasm
Cytoskeleton
Direct protein sequencing
Disease variant
Epidermolysis bullosa
Limb-girdle muscular dystrophy
Methylation
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
SH3 domain
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
namePLEC
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8960164] ENSEMBL:ENSG00000178209 PLEC [Homo sapiens]
secondaryIdentifierPLEC_HUMAN
Q15148
Q16640
Q6S376
Q6S377
Q6S378
Q6S379
Q6S380
Q6S381
Q6S382
Q6S383
sequenceLength4684
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:152218] UniProt:Q15149-2 PLEC [Homo sapiens]
[ReferenceIsoform:152219] UniProt:Q15149-3 PLEC [Homo sapiens]
[ReferenceIsoform:243204] UniProt:Q15149-4 PLEC [Homo sapiens]
[ReferenceIsoform:405828] UniProt:Q15149-1 PLEC [Homo sapiens]
[ReferenceIsoform:423420] UniProt:Q15149-5 PLEC [Homo sapiens]
[ReferenceIsoform:423421] UniProt:Q15149-6 PLEC [Homo sapiens]
[ReferenceIsoform:423422] UniProt:Q15149-7 PLEC [Homo sapiens]
[ReferenceIsoform:423423] UniProt:Q15149-8 PLEC [Homo sapiens]
[ReferenceIsoform:423424] UniProt:Q15149-9 PLEC [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:201575] PLEC [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:350618] PLEC(2396-4684) [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:350638] PLEC(1-2395) [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:8960657] PLEC [extracellular region] [Homo sapiens]
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