Amish, Mennonite, and Hutterite
Genetic Disorder Database

Knobloch syndrome

Disorder
OMIM #: #267750  (Click to access OMIM database)
Disorder: Knobloch syndrome 
Also known as: KNO
RETINAL DETACHMENT AND OCCIPITAL ENCEPHALOCELE 
Clinical
Phenotype: high myopia, cataracts, dislocated lens, vitreoretinal degeneration, retinal detachment, other ocular abnormalities, occipital skull defects, occipital encephalocele, occipital dermal sinus tract 
Seen In: Amish
Old Order Mennonite
Old Colony Mennonite
Unknown/Other Mennonite
Hutterite
Remarks: Autosomal recessive  
Mutations
1   Amish  
Gene: COL18A1
Base Change: c.3522_3523delCT
Amino Acid Change: p.Leu1175Valfs*72
Last updated: 2022-12-07 

References
Personal communication; seen at CSC.  

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