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Metadata
ID DOID:0110332
Name Leber congenital amaurosis 4
Definition A Leber congenital amaurosis that is characterized by a relatively severe phenotype, with maculopathy and marked bone-spicule pigmentary retinopathy in most and keratoconus and cataract in a large subset and that has_material_basis_in mutation in the AIPL1 gene on chromosome 17p13.
https://www.ncbi.nlm.nih.gov/pubmed/10615133
Xrefs

ICD10CM:H35.5

MESH:C565778

MIM:604393

Synonyms

LCA4 [EXACT]

Parent Relationships

is_a Leber congenital amaurosis

is_a autosomal recessive disease

Subclass Logical Relationships

has phenotype some Retinopathy

has material basis in some autosomal recessive inheritance

disease has basis in some Abnormality of prenatal development or birth

has phenotype some Keratoconus

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