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Details on Person UniProt:Q4AC94 C2CD3

Class:IdReferenceGeneProduct:220573
_chainChangeLogchain:1-2353 added on Fri February 6 2015
_displayNameUniProt:Q4AC94 C2CD3
_timestamp2024-11-03 20:18:10
chainchain:1-2353
checksumD559291AEB1B0DA1
commentFUNCTION Component of the centrioles that acts as a positive regulator of centriole elongation (PubMed:24997988). Promotes assembly of centriolar distal appendage, a structure at the distal end of the mother centriole that acts as an anchor of the cilium, and is required for recruitment of centriolar distal appendages proteins CEP83, SCLT1, CEP89, FBF1 and CEP164. Not required for centriolar satellite integrity or RAB8 activation. Required for primary cilium formation (PubMed:23769972). Required for sonic hedgehog/SHH signaling and for proteolytic processing of GLI3.SUBUNIT Interacts with IFT88, BBS4 and PCM1 (By similarity). Interacts with OFD1; OFD1 may act as a negative regulator of C2CD3. Associates with the BBSome complex.INTERACTION Localizes to centrioles and procentrioles both in interphase and mitosis. Localizes to centriolar satellites, localization is dependent on PCM1 and dynein-mediated retrograde transport. Also localizes to the distal ends of the mother and daughter centrioles.ALTERNATIVE PRODUCTS The disease is caused by variants affecting the gene represented in this entry.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: C2 domain-containing protein 3
geneNameC2CD3
identifierQ4AC94
isSequenceChangedFALSE
keywordAlternative splicing
Cell projection
Ciliopathy
Cilium biogenesis/degradation
Cytoplasm
Cytoskeleton
Disease variant
Intellectual disability
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameC2CD3
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:9002228] ENSEMBL:ENSG00000168014 C2CD3 [Homo sapiens]
secondaryIdentifierC2CD3_HUMAN
C9JR55
E2QRD1
Q2NLE1
Q3C1U9
Q6ZU92
Q8IYM4
Q8NB87
Q8NDH7
Q9Y4M2
sequenceLength2353
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:220574] UniProt:Q4AC94-2 C2CD3 [Homo sapiens]
[ReferenceIsoform:220575] UniProt:Q4AC94-3 C2CD3 [Homo sapiens]
[ReferenceIsoform:220576] UniProt:Q4AC94-4 C2CD3 [Homo sapiens]
[ReferenceIsoform:384571] UniProt:Q4AC94-5 C2CD3 [Homo sapiens]
[ReferenceIsoform:406847] UniProt:Q4AC94-1 C2CD3 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:5620926] C2CD3 [cytosol] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q4AC94 C2CD3 (220573)