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ProteinQ21118898· pop 5· linked from 62 articles

Also known as Joubert syndrome type 10, protein 71-7A, oral-facial-digital syndrome 1 protein, OFD1 centriole and centriolar satellite protein

Oral-facial-digital syndrome 1 protein is a protein that in humans is encoded by the OFD1 gene.

Protein · UniProt

Centriole and centriolar satellite protein OFD1

Gene
OFD1
Organism
Homo sapiens (Human)
Length
1,012 aa
Molecular mass
116,671 Da
Evidence
1: Evidence at protein level

Component of the centrioles controlling mother and daughter centrioles length. Recruits to the centriole IFT88 and centriole distal appendage-specific proteins including CEP164 (By similarity). Involved in the biogenesis of the cilium, a centriole-associated function. The cilium is a cell surface projection found in many vertebrate cells required to transduce signals important for development and tissue homeostasis (PubMed:33934390). Plays an important role in development by regulating Wnt signaling and the specification of the left-right axis. Only OFD1 localized at the centriolar satellit…

Alternative splicingCell projectionCiliopathyCiliumCilium biogenesis/degradationCoiled coilCytoplasmCytoskeleton
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Oral-facial-digital syndrome 1 protein is a protein that in humans is encoded by the OFD1 gene.

Human chromosomal region Xp22.3-p21.3 comprises the area between the pseudoautosomal boundary and the Duchenne muscular dystrophy gene (MIM 300377). This region harbors several disease loci, including OFD1 (MIM 311200), CFNS (MIM 304110), DFN6 (MIM 300066), and SEDT (MIM 313400). It also contains a region of homology with both the short and the long arms of the Y chromosome and undergoes frequent chromosomal rearrangements.[supplied by OMIM]

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