Reactome | FH [mitochondrial matrix]

FH [mitochondrial matrix]

Stable Identifier

R-HSA-70980

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

fumarate hydratase, Fumarate hydratase, mitochondrial, Fumarase

Locations in the PathwayBrowser

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External Reference Information

External Reference

UniProt:P07954 FH

Gene Names

Chain

initiator methionine:, transit peptide:1-44, chain:45-510

Reference Genes

BioGPS Gene:2271 FH

COSMIC (genes):FH FH

CTD Gene:2271 FH

dbSNP Gene:2271 FH

ENSEMBL:ENSG00000091483 FH

ENSEMBL_homo_sapiens_GENE:ENSG00000091483.8 FH

HGNC:3700 FH

KEGG Gene (Homo sapiens):2271 FH

Monarch:2271 FH

NCBI Gene:2271 FH

OMIM:136850 FH

UCSC:P07954 FH

Reference Transcript

RefSeq:NM_000143.3 FH

Other Identifiers

11744166_at

16701077

203032_PM_s_at

203032_s_at

203033_PM_x_at

203033_x_at

214170_PM_x_at

214170_x_at

2271

2463439

2463440

2463441

2463443

2463444

2463445

2463446

2463447

2463449

2463450

2463454

2463455

2463458

2463460

2463461

2852750

32546_at

7925480

A_23_P34733

GE59264

GO:0003824

GO:0004333

GO:0005515

GO:0005576

GO:0005615

GO:0005634

GO:0005694

GO:0005737

GO:0005739

GO:0005759

GO:0005829

GO:0006091

GO:0006099

GO:0006106

GO:0006108

GO:0006281

GO:0006974

GO:0016829

GO:0043226

GO:0045239

GO:0048873

GO:0070062

GO:0120162

HMNXSV003025621

Hs.75653.0.A1_3p_a_at

Hs.75653.1.A1_3p_s_at

ILMN_1719392

PH_hs_0024542

TC01004025.hg

U59309_at

Participates

as a member of

as a component of

FH tetramer [mitochondrial matrix] (Homo sapiens)

Inferred To

FH [mitochondrial matrix] (Bos taurus)

FH [mitochondrial matrix] (Caenorhabditis elegans)

FH [mitochondrial matrix] (Canis familiaris)

FH [mitochondrial matrix] (Danio rerio)

FH [mitochondrial matrix] (Dictyostelium discoideum)

FH [mitochondrial matrix] (Gallus gallus)

FH [mitochondrial matrix] (Mus musculus)

FH [mitochondrial matrix] (Rattus norvegicus)

FH [mitochondrial matrix] (Saccharomyces cerevisiae)

FH [mitochondrial matrix] (Schizosaccharomyces pombe)

FH [mitochondrial matrix] (Sus scrofa)

Fum1 [mitochondrial matrix] (Drosophila melanogaster)

Cross References

RefSeq

NP_000134.2

OpenTargets

ENSG00000091483

GeneCards

P07954

HPA

ENSG00000091483-FH

Ensembl

ENSG00000091483, ENSP00000355518, ENST00000366560

PRO

P07954

Pharos - Targets

P07954

Orphanet

16049

HMDB Protein

HMDBP00861

PDB

5UPP, 6VBE, 6EBT, 5D6B, 7LUB, 3E04, 6V8F

Interactors (6)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:P21673 SAT1	1	SAT1 [cytosol] (R-HSA-351236)	0.556	3
UniProt:Q6DKK2 TTC19	1	TTC19 [mitochondrial inner membrane] (R-HSA-9906047)	0.556	3
UniProt:O15160 POLR1C	2	POLR1C [nucleoplasm] (R-HSA-63498) POLR1C [cytosol] (R-HSA-1964454)	0.556	3
UniProt:P11309 PIM1	1	PIM1 [nucleoplasm] (R-HSA-3215094)	0.556	3
UniProt:Q9NQT4 EXOSC5	2	EXOSC5 [cytosol] (R-HSA-429889) EXOSC5 [nucleoplasm] (R-HSA-6791550)	0.556	3
UniProt:P13569 CFTR	17	PolyUb-CFTR [clathrin-coated endocytic vesicle membrane] (R-HSA-8869104) PolyUb-CFTR [plasma membrane] (R-HSA-8867595) CFTR [lysosomal membrane] (R-HSA-5627279) CFTR [Golgi-associated vesicle membrane] (R-HSA-5627079) CFTR [endosome membrane] (R-HSA-6782965) PolyUb-CFTR [endosome membrane] (R-HSA-6782976) CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866834) ub-CFTR F508del [cytosol] (R-HSA-8866838) ub-CFTR F508del [endoplasmic reticulum membrane] (R-HSA-8866842) CFTR G542* [plasma membrane] (R-HSA-5678986) CFTR G551D [plasma membrane] (R-HSA-5678977) CFTR W1282* [plasma membrane] (R-HSA-5678976) CFTR F508del [plasma membrane] (R-HSA-5678978) CFTR N1303K [plasma membrane] (R-HSA-5678983) CFTR [plasma membrane] (R-HSA-383188) CFTR [endoplasmic reticulum membrane] (R-HSA-8866837) misfolded CFTR [endoplasmic reticulum membrane] (R-HSA-8868730)	0.483	5

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