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Metadata
ID DOID:0070735
PURL http://purl.obolibrary.org/obo/DOID_0070735 Copy
Name auditory neuropathy and optic atrophy
Definition A multiple mitochondrial dysfunctions syndrome characterized by bilateral auditory neuropathy and optic atrophy that has_material_basis_in homozygous or compound heterozygous mutation in the FDXR gene on chromosome 17q25.
https://pubmed.ncbi.nlm.nih.gov/28965846/
Xrefs

GARD:0017983

MIM:617717

ORDO:542585

SNOMEDCT_US_2026_03_01:1222649004

SKOS

exactMatch UMLS_CUI:C4521678

exactMatch GARD:0017983

exactMatch MIM:617717

exactMatch ORDO:542585

Subsets

DO_rare_slim

Synonyms

MMDS9A [EXACT]

multiple mitochondrial dysfunctions syndrome 9A [EXACT]

Parent Relationships

is_a multiple mitochondrial dysfunctions syndrome

is_a vestibulocochlear nerve disease

is_a optic nerve disease

is_a autosomal recessive disease

Subclass Logical Relationships

disease has location some cranial nerve II

has material basis in some autosomal recessive inheritance

disease has location some vestibulocochlear nerve

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