Q60HF9 (FUMH_MACFA) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Fumarate hydratase, mitochondrial
Short name=Fumarase
EC=4.2.1.2

Gene names

Name:	FH
ORF Names:	QorA-13820

Organism

Macaca fascicularis (Crab-eating macaque) (Cynomolgus monkey)

Taxonomic identifier

9541 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Cercopithecoidea › Cercopithecidae › Cercopithecinae › Macaca

Protein attributes

Sequence length	510 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at transcript level

General annotation (Comments)

Catalytic activity	(S)-malate = fumarate + H2O.
Pathway	Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
Subunit structure	Homotetramer By similarity.
Subcellular location	Isoform Mitochondrial: Mitochondrion By similarity. Isoform Cytoplasmic: Cytoplasm By similarity.
Post-translational modification	Isoform Cytoplasmic contains is acetylated at position 2 By similarity.
Miscellaneous	There are 2 substrate binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors By similarity.
Sequence similarities	Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.

Ontologies

Keywords
Biological process	Tricarboxylic acid cycle
Cellular component	Cytoplasm Mitochondrion
Coding sequence diversity	Alternative initiation
Domain	Transit peptide
Molecular function	Lyase
PTM	Acetylation
Gene Ontology (GO)
Biological_process	fumarate metabolic process Inferred from electronic annotation. Source: InterPro tricarboxylic acid cycle Inferred from electronic annotation. Source: UniProtKB-UniPathway
Cellular_component	mitochondrion Inferred from electronic annotation. Source: UniProtKB-SubCell tricarboxylic acid cycle enzyme complex Inferred from electronic annotation. Source: InterPro
Molecular_function	fumarate hydratase activity Inferred from electronic annotation. Source: EC
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative initiation. [Align] [Select]

Isoform Mitochondrial (identifier: Q60HF9-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform Cytoplasmic (identifier: Q60HF9-2)

The sequence of this isoform differs from the canonical sequence as follows:
     1-43: Missing.

Note: Initiator Met-1 is removed. Contains a N-acetylalanine at position 2 (By similarity).

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 44	44	Mitochondrion By similarity
	Chain	45 – 510	466	Fumarate hydratase, mitochondrial		PRO_0000010321
Regions
	Region	176 – 179	4	B site By similarity
	Region	186 – 188	3	Substrate binding By similarity
Sites
	Binding site	147	1	Substrate By similarity
Amino acid modifications
	Modified residue	66	1	N6-acetyllysine By similarity
	Modified residue	80	1	N6-acetyllysine By similarity
	Modified residue	94	1	N6-acetyllysine By similarity
	Modified residue	115	1	N6-acetyllysine By similarity
	Modified residue	256	1	N6-acetyllysine By similarity
	Modified residue	292	1	N6-acetyllysine By similarity
	Modified residue	477	1	N6-acetyllysine By similarity
Natural variations
	Alternative sequence	1 – 43	43	Missing in isoform Cytoplasmic.		VSP_018966

Sequences

	Sequence		Length	Mass (Da)
	Isoform Mitochondrial [UniParc]. Last modified November 23, 2004. Version 1. Checksum: F5FC970E977DEA09	FASTA	510	54,766
	10 20 30 40 50 60 MYRALWLLAR SRRLVRPPAS ALASAPGLSG AAVPSFWPPN AARMASQNSF RIEYDTFGEL 70 80 90 100 110 120 KVPNDKYYGA QTVRSTMNFK IGGVTERMPT PVIKAFGILK RAAAEVNQDY GLDPKIANAI 130 140 150 160 170 180 MKAADEVAEG KLNDHFPLVV WQTGSGTQTN MNVNEVISNR AIEMLGGELG SKIPVHPNDH 190 200 210 220 230 240 VNKSQSSNDT FPTAMHIAAA IEVHEVLLPG LQKLHDALDA KSKEFAQIIK IGRTHTQDAV 250 260 270 280 290 300 PLTLGQEFSG YVQQVKYAVT RIKAAMPRIY ELAAGGTAVG TGLNTRIGFA EKVAAKVAAL 310 320 330 340 350 360 TGLPFVTAPN KFEALAAHDA LVELSGAMNT TACSLMKIAN DIRFLGSGPR SGLGELILPE 370 380 390 400 410 420 NEPGSSIMPG KVNPTQCEAM TMVAAQVMGN HVAVTVGGSN GHFELNVFKP MMIKNVLHSA 430 440 450 460 470 480 RLLGDASVSF TENCVVGIQA NTERINKLMN ESLMLVTALN PHIGYDKAAK IAKTAHKNGS 490 500 510 TLKETAIELG YLTAEQFDEW VKPKDMLGPK
	Isoform Cytoplasmic [UniParc]. Checksum: 291AC7968C06254F	FASTA	467	50,181
	10 20 30 40 50 60 MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA 70 80 90 100 110 120 AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE 130 140 150 160 170 180 MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK 190 200 210 220 230 240 EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAVTRIK AAMPRIYELA AGGTAVGTGL 250 260 270 280 290 300 NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR 310 320 330 340 350 360 FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF 370 380 390 400 410 420 ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI 430 440 450 460 GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK

References

[1]	"Isolation and characterization of cDNA for macaque neurological disease genes." Kusuda J., Osada N., Tanuma R., Hirata M., Sugano S., Hashimoto K. Submitted (OCT-2003) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Occipital cortex.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AB125168 mRNA. Translation: BAD51956.1.
3D structure databases
ProteinModelPortal	Q60HF9.
SMR	Q60HF9. Positions 49-510.
ModBase	Search...
Proteomic databases
PRIDE	Q60HF9.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Phylogenomic databases
HOVERGEN	HBG002183.
Enzyme and pathway databases
UniPathway	UPA00223; UER01007.
Family and domain databases
Gene3D	1.10.275.10. 1 hit.
InterPro	IPR005677. Fum_hydII. IPR024083. Fumarase/histidase_N. IPR018951. Fumarase_C_C. IPR000362. Fumarate_lyase. IPR020557. Fumarate_lyase_CS. IPR022761. Fumarate_lyase_N. IPR008948. L-Aspartase-like. [Graphical view]
Pfam	PF10415. FumaraseC_C. 1 hit. PF00206. Lyase_1. 1 hit. [Graphical view]
PRINTS	PR00149. FUMRATELYASE.
SUPFAM	SSF48557. L-Aspartase-like. 1 hit.
TIGRFAMs	TIGR00979. fumC_II. 1 hit.
PROSITE	PS00163. FUMARATE_LYASES. 1 hit. [Graphical view]
ProtoNet	Search...

Entry information

Entry name

FUMH_MACFA

Accession

Primary (citable) accession number: Q60HF9

Entry history

Integrated into UniProtKB/Swiss-Prot:	April 12, 2005
Last sequence update:	November 23, 2004
Last modified:	March 6, 2013
This is version 55 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program