OFD1 (OFD1 centriole and centriolar satellite protein)

2014-11-01  

Identity

HGNC
LOCATION
Xp22.2
LOCUSID
ALIAS
71-7A,CXorf5,JBTS10,RP23,SGBS2
FUSION GENES

Other Information

Locus ID:

NCBI: 8481
MIM: 300170
HGNC: 2567
Ensembl: ENSG00000046651

Variants:

dbSNP: 8481
ClinVar: 8481
TCGA: ENSG00000046651
COSMIC: OFD1

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000046651ENST00000340096O75665
ENSG00000046651ENST00000340096E9KL37
ENSG00000046651ENST00000380550O75665
ENSG00000046651ENST00000380567A6NF31
ENSG00000046651ENST00000398395A0A0A0MS89

Expression (GTEx)

0
5
10
15
20
25
30
35
40
45
50

Pathways

PathwaySourceExternal ID
Organelle biogenesis and maintenanceREACTOMER-HSA-1852241
Cilium AssemblyREACTOMER-HSA-5617833
Anchoring of the basal body to the plasma membraneREACTOMER-HSA-5620912
Signal TransductionREACTOMER-HSA-162582
Signaling by HedgehogREACTOMER-HSA-5358351
Hedgehog 'off' stateREACTOMER-HSA-5610787
Cell CycleREACTOMER-HSA-1640170
Cell Cycle, MitoticREACTOMER-HSA-69278
Mitotic G2-G2/M phasesREACTOMER-HSA-453274
G2/M TransitionREACTOMER-HSA-69275
Regulation of PLK1 Activity at G2/M TransitionREACTOMER-HSA-2565942
Centrosome maturationREACTOMER-HSA-380287
Recruitment of mitotic centrosome proteins and complexesREACTOMER-HSA-380270
Loss of proteins required for interphase microtubule organization from the centrosomeREACTOMER-HSA-380284
Loss of Nlp from mitotic centrosomesREACTOMER-HSA-380259
AURKA Activation by TPX2REACTOMER-HSA-8854518

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
381393552023Extraciliary OFD1 Is Involved in Melanocyte Survival through Cell Adhesion to ECM via Paxillin.0
381393552023Extraciliary OFD1 Is Involved in Melanocyte Survival through Cell Adhesion to ECM via Paxillin.0
329447892021A rare mutant of OFD1 gene responsible for Joubert syndrome with significant phenotype variation.8
342416342021A ciliopathy complex builds distal appendages to initiate ciliogenesis.19
329447892021A rare mutant of OFD1 gene responsible for Joubert syndrome with significant phenotype variation.8
342416342021A ciliopathy complex builds distal appendages to initiate ciliogenesis.19
324737062020The centrosomal/basal body protein OFD1 is required for microtubule organization and cell cycle progression.9
326777602020Indian child with novel variant in OFD1 gene.0
324737062020The centrosomal/basal body protein OFD1 is required for microtubule organization and cell cycle progression.9
326777602020Indian child with novel variant in OFD1 gene.0
304019172019Clinical spectrum of male patients with OFD1 mutations.6
308957202019A novel pathogenic variant in OFD1 results in X-linked Joubert syndrome with orofaciodigital features and pituitary aplasia.7
313666082019Truncating mutations in exons 20 and 21 of OFD1 can cause primary ciliary dyskinesia without associated syndromic symptoms.19
313731792019The expanding phenotype of OFD1-related disorders: Hemizygous loss-of-function variants in three patients with primary ciliary dyskinesia.20
304019172019Clinical spectrum of male patients with OFD1 mutations.6

Citation

Dessen P

OFD1 (OFD1 centriole and centriolar satellite protein)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/71100/gene-fusions/teaching-explorer/