VARIANT: 2271v1
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Entry
2271v1 Variant
Name
FH deficiency
Type
Loss of function
Gene
FH
fumarate hydratase [KO:
K01679
]
Organism
hsa_var Human gene variants (Homo sapiens)
Variation
mutations
OmimVar:
136850
Network
nt06031
Citrate cycle and pyruvate metabolism
Disease
H02004
Fumarase deficiency
Reference
PMID:
8200987
Authors
Bourgeron T, Chretien D, Poggi-Bach J, Doonan S, Rabier D, Letouze P, Munnich A, Rotig A, Landrieu P, Rustin P
Title
Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency.
Journal
J Clin Invest 93:2514-8 (1994)
DOI:
10.1172/JCI117261
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