Gene Information
Gene NameFH
OrganismHomo sapiens (Human)
Gene Length510
Protein NamesFumarate hydratase, mitochondrial (Fumarase) (HsFH) (EC 4.2.1.2)
Target NameNA
Target TypeNA
Gene Age>4290 ( ENSG00000091483 )
Evolutionary StageCellular_organisms
AlphaFoldDBP07954
Gene CardFH
Uniprot IDP07954
PfamPF10415; PF00206
In ASDno
Allosteric Predictionyes (0.543012701)
Ortholog in AnimalsAlpaca_ FH ( ortholog_one2one )
Dog_ FH ( ortholog_one2one )
GuineaPig_ FH ( ortholog_one2one )
Macaque_ FH ( ortholog_one2one )
Mouse_ Fh1 ( ortholog_one2one )
Pig_ FH ( ortholog_one2one )
Rat_ Fh ( ortholog_one2one )
OhnologsNA
ParalogsADSL, ASL
TissueNA
Tissue SpecificityExpressed in red blood cells;
underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. {ECO:0000269|PubMed:22509282}.
Pharmacological Animal ModelsNULL(Chimpanzee);NULL(Mouse);0.026335(Rat);0.180889(Rabbit)
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 3E04  (X-ray  1.95A  5D6B  (X-ray  2.10A  5UPP  (X-ray  1.80A  6EBT  (X-ray  2.30A  6V8F  (X-ray  2.30A  6VBE  (X-ray  1.90A  7LUB  (X-ray  2.15A 
DBSNP rs121913121  rs886039363  rs863224015  rs779707997  rs752232718  rs121913123  rs935002190  rs121913118  rs1553341046  rs121913119 
Subcellular location [CC][Isoform Mitochondrial]: Mitochondrion {ECO:0000269|PubMed:27037871}.;
[Isoform Cytoplasmic]: Cytoplasm, cytosol {ECO:0000269|PubMed:20231875, ECO:0000269|PubMed:22509282, ECO:0000269|PubMed:26237645, ECO:0000269|PubMed:27037871}. Nucleus {ECO:0000269|PubMed:20231875, ECO:0000269|PubMed:26237645}. Chromosome {ECO:0000269|PubMed:26237645}. Note=Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs) following phosphorylation by PRKDC. {ECO:0000269|PubMed:26237645}.
Mouse PG classificationNA
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