Joubert syndrome
Gene: OFD1EnsemblGeneIds (GRCh38): ENSG00000046651
EnsemblGeneIds (GRCh37): ENSG00000046651
OMIM: 300170, Gene2Phenotype
OFD1 is in 26 panels
1 review
Penny Clouston (Oxford)
On current diagnostic panel; positive families within patient cohort. Evidence from literature.Created: 16 Mar 2016, 9:07 a.m.
Mode of inheritance
X-LINKED: hemizygous mutation in males, biallelic mutations in females
Phenotypes
X-linked Joubert syndrome; Oral-facial-digital syndrome I
Publications
Variants in this GENE are reported as part of current diagnostic practice
Details
- Mode of Inheritance
- Other - please specifiy in evaluation comments
- Sources
-
- Phenotypes
-
- X-linked Joubert syndrome
- Oral-facial-digital syndrome I
- OMIM
- 300170
- Clinvar variants
- Variants in OFD1
- Penetrance
- Complete
- Publications
- Panels with this gene
-
- Pigmentary skin disorders
- Ophthalmological ciliopathies
- Skeletal dysplasia
- Deafness and congenital structural abnormalities
- Retinal disorders
- Neurological ciliopathies
- Limb disorders
- Dystonia, chorea or related movement disorder, childhood onset
- Osteogenesis imperfecta
- Intellectual disability
- Beckwith-Wiedemann syndrome (BWS) and other congenital overgrowth disorders
- Ocular coloboma
- Hydrocephalus
- Renal ciliopathies
- Clefting
- Unexplained kidney failure in young people
- Ductal plate malformation
- Fetal anomalies
- Structural eye disease
- Rare multisystem ciliopathy disorders
- DDG2P
- Respiratory ciliopathies including non-CF bronchiectasis
- Glaucoma (developmental)
- Thoracic dystrophies
- Primary ciliary disorders
- Cystic kidney disease
History Filter Activity
Approved Gene
Alice Gardham (Genomics England)This proposed gene was validated and added to this panel
Created
Penny Clouston (Oxford)OFD1 was created by PennyC
Added New Source
Penny Clouston (Oxford)OFD1 was added to Joubert syndromepanel. Sources: Expert Review