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Details on Person UniProt:Q14031 COL4A6

Class:IdReferenceGeneProduct:51188
_chainChangeLogsignal peptide:1-22 added on Fri February 6 2015;chain:23-1691 added on Fri February 6 2015
_displayNameUniProt:Q14031 COL4A6
_timestamp2024-11-03 20:09:25
chainsignal peptide:1-22
chain:23-1691
checksum9313294D4CE63067
commentFUNCTION Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen.SUBUNIT There are six type IV collagen isoforms, alpha 1(IV)-alpha 6(IV), each of which can form a triple helix structure with 2 other chains to generate type IV collagen network.INTERACTION Alpha chains of type IV collagen have a non-collagenous domain (NC1) at their C-terminus, frequent interruptions of the G-X-Y repeats in the long central triple-helical domain (which may cause flexibility in the triple helix), and a short N-terminal triple-helical 7S domain.PTM Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.PTM Type IV collagens contain numerous cysteine residues which are involved in inter- and intramolecular disulfide bonding. 12 of these, located in the NC1 domain, are conserved in all known type IV collagens.PTM The trimeric structure of the NC1 domains is stabilized by covalent bonds between Lys and Met residues.DISEASE Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS).DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the type IV collagen family.
descriptionrecommendedName: Collagen alpha-6(IV) chain
geneNameCOL4A6
identifierQ14031
isSequenceChangedFALSE
keywordAlternative splicing
Basement membrane
Cell adhesion
Chromosomal rearrangement
Collagen
Deafness
Disease variant
Disulfide bond
Extracellular matrix
Glycoprotein
Hydroxylation
Non-syndromic deafness
Proteomics identification
Reference proteome
Repeat
Secreted
Signal
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameCOL4A6
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:4793748] ENSEMBL:ENSG00000197565 COL4A6 [Homo sapiens]
secondaryIdentifierCO4A6_HUMAN
Q12823
Q14053
Q5JYH6
Q5JYH8
Q9NQM5
Q9NTX3
Q9UJ76
Q9UMG6
Q9Y4L4
sequenceLength1691
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:144883] UniProt:Q14031-2 COL4A6 [Homo sapiens]
[ReferenceIsoform:405684] UniProt:Q14031-1 COL4A6 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:1980196] COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2022125] 3x4Hyp-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2022447] 5Hyl-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2022486] 3x4Hyp-3Hyp-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2022499] 3Hyp-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2022969] 3x4Hyp-3Hyp-5Hyl-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2022979] 3x4Hyp-5Hyl-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2023535] GalHyl-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2023561] 3x4Hyp-3Hyp-GalHyl-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
[EntityWithAccessionedSequence:2023563] 3x4Hyp-GalHyl-COL4A6 [endoplasmic reticulum lumen] [Homo sapiens]
List all 52 refering instances
(referenceSequence)[ModifiedResidue:2022117] (2S,4R)-4-hydroxyproline at unknown position
[ModifiedResidue:2022498] (2S,5R)-5-hydroxylysine at unknown position
[ModifiedResidue:2022515] (2S,3S)-3-hydroxyproline at unknown position
[ModifiedResidue:2022941] (2S,5R)-5-hydroxylysine at unknown position
[ModifiedResidue:2023536] O5-galactosyl-L-hydroxylysine at unknown position
[ModifiedResidue:2023616] O5-glucosylgalactosyl-L-hydroxylysine at unknown position
[GroupModifiedResidue:2682372] (2S,5R)-5-hydroxylysine (sulfilimine bond) at unknown position
[GroupModifiedResidue:2682399] (2S,5R)-5-hydroxylysine (sulfilimine bond) at unknown position
[GroupModifiedResidue:2682408] (2S,5R)-5-hydroxylysine (sulfilimine bond) at unknown position
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No pathways have been reviewed or authored by UniProt:Q14031 COL4A6 (51188)