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Protein

Fumarate hydratase, mitochondrial

Gene

Fh

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalytic activityi

(S)-malate = fumarate + H2O.

Pathwayi: tricarboxylic acid cycle

This protein is involved in step 1 of the subpathway that synthesizes (S)-malate from fumarate.
Proteins known to be involved in this subpathway in this organism are:
  1. Fumarate hydratase, mitochondrial (Fh)
This subpathway is part of the pathway tricarboxylic acid cycle, which is itself part of Carbohydrate metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes (S)-malate from fumarate, the pathway tricarboxylic acid cycle and in Carbohydrate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei144SubstrateBy similarity1

GO - Molecular functioni

  • fumarate hydratase activity Source: RGD

GO - Biological processi

  • fumarate metabolic process Source: RGD
  • malate metabolic process Source: RGD
  • protein tetramerization Source: GO_Central
  • tricarboxylic acid cycle Source: RGD
Complete GO annotation...

Keywords - Molecular functioni

Lyase

Keywords - Biological processi

Tricarboxylic acid cycle

Enzyme and pathway databases

SABIO-RKP14408.
UniPathwayiUPA00223; UER01007.

Names & Taxonomyi

Protein namesi
Recommended name:
Fumarate hydratase, mitochondrial (EC:4.2.1.2)
Short name:
Fumarase
Gene namesi
Name:Fh
Synonyms:Fh1
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi2614. Fh.

Subcellular locationi

GO - Cellular componenti

  • cytosol Source: GO_Central
  • mitochondrion Source: RGD
  • tricarboxylic acid cycle enzyme complex Source: InterPro
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 41MitochondrionAdd BLAST41
ChainiPRO_000001032542 – 507Fumarate hydratase, mitochondrialAdd BLAST466
Isoform Cytoplasmic (identifier: P14408-2)
Initiator methionineiRemovedBy similarity

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei58N6-acetyllysine; alternateBy similarity1
Modified residuei58N6-succinyllysine; alternateBy similarity1
Modified residuei63N6-acetyllysine; alternateBy similarity1
Modified residuei63N6-succinyllysine; alternateBy similarity1
Modified residuei77N6-acetyllysine; alternateBy similarity1
Modified residuei77N6-succinyllysine; alternateBy similarity1
Modified residuei82PhosphothreonineBy similarity1
Modified residuei91N6-acetyllysineBy similarity1
Modified residuei112N6-acetyllysine; alternateBy similarity1
Modified residuei112N6-succinyllysine; alternateBy similarity1
Modified residuei119N6-acetyllysine; alternateBy similarity1
Modified residuei119N6-succinyllysine; alternateBy similarity1
Modified residuei210N6-acetyllysineBy similarity1
Modified residuei220N6-acetyllysine; alternateBy similarity1
Modified residuei220N6-succinyllysine; alternateBy similarity1
Modified residuei289N6-acetyllysine; alternateBy similarity1
Modified residuei289N6-succinyllysine; alternateBy similarity1
Modified residuei363PhosphoserineBy similarity1
Modified residuei464N6-succinyllysineBy similarity1
Modified residuei470N6-succinyllysineBy similarity1
Modified residuei499N6-acetyllysineBy similarity1
Isoform Cytoplasmic (identifier: P14408-2)
Modified residuei2N-acetylalanine By similarityBy similarity1

Post-translational modificationi

Isoform Cytoplasmic is acetylated at position 2.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

PaxDbiP14408.
PeptideAtlasiP14408.
PRIDEiP14408.

PTM databases

iPTMnetiP14408.
PhosphoSitePlusiP14408.

Interactioni

Subunit structurei

Homotetramer.

Protein-protein interaction databases

IntActiP14408. 1 interactor.
STRINGi10116.ENSRNOP00000004917.

Structurei

3D structure databases

ProteinModelPortaliP14408.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni173 – 176B siteBy similarity4
Regioni183 – 185Substrate bindingBy similarity3

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1317. Eukaryota.
COG0114. LUCA.
HOGENOMiHOG000061736.
HOVERGENiHBG002183.
InParanoidiP14408.
PhylomeDBiP14408.

Family and domain databases

Gene3Di1.10.275.10. 1 hit.
HAMAPiMF_00743. FumaraseC. 1 hit.
InterProiIPR005677. Fum_hydII.
IPR024083. Fumarase/histidase_N.
IPR018951. Fumarase_C_C.
IPR020557. Fumarate_lyase_CS.
IPR000362. Fumarate_lyase_fam.
IPR022761. Fumarate_lyase_N.
IPR008948. L-Aspartase-like.
[Graphical view]
PANTHERiPTHR11444. PTHR11444. 1 hit.
PfamiPF10415. FumaraseC_C. 1 hit.
PF00206. Lyase_1. 1 hit.
[Graphical view]
PRINTSiPR00149. FUMRATELYASE.
SUPFAMiSSF48557. SSF48557. 1 hit.
TIGRFAMsiTIGR00979. fumC_II. 1 hit.
PROSITEiPS00163. FUMARATE_LYASES. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative initiation. AlignAdd to basket

Isoform Mitochondrial (identifier: P14408-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MNRAFCLLAR SRRFPRVPSA GAVLSGEAAT LPRCAPNVVR MASQNSFRIE
60 70 80 90 100
YDTFGELKVP TDKYYGAQTV RSTMNFKIGG ATERMPIPVI KAFGILKRAA
110 120 130 140 150
AEVNQEYGLD PKIASAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV
160 170 180 190 200
NEVISNRAIE MLGGELGSKK PVHPNDHVNK SQSSNDTFPT AMHIAAALEV
210 220 230 240 250
HQVLLPGLQK LHDALSAKSK EFAQVIKIGR THTQDAVPLT LGQEFSGYVQ
260 270 280 290 300
QVQYAMERIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL
310 320 330 340 350
PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL
360 370 380 390 400
GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF
410 420 430 440 450
ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL
460 470 480 490 500
MLVTALNPHI GYDKAAKIAK TAHKNGSTLK KTAIELGYLT AEQFDEWVKP

KDMLGPK
Length:507
Mass (Da):54,464
Last modified:January 1, 1990 - v1
Checksum:i0304255E04C0D7D8
GO
Isoform Cytoplasmic (identifier: P14408-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-40: Missing.

Show »
Length:467
Mass (Da):50,170
Checksum:i33CC61C8CC024663
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0189681 – 40Missing in isoform Cytoplasmic. CuratedAdd BLAST40

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04473 mRNA. Translation: AAA41177.1.
PIRiA31424. UFRT.
UniGeneiRn.29782.

Genome annotation databases

UCSCiRGD:2614. rat. [P14408-1]

Keywords - Coding sequence diversityi

Alternative initiation

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04473 mRNA. Translation: AAA41177.1.
PIRiA31424. UFRT.
UniGeneiRn.29782.

3D structure databases

ProteinModelPortaliP14408.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP14408. 1 interactor.
STRINGi10116.ENSRNOP00000004917.

PTM databases

iPTMnetiP14408.
PhosphoSitePlusiP14408.

Proteomic databases

PaxDbiP14408.
PeptideAtlasiP14408.
PRIDEiP14408.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

UCSCiRGD:2614. rat. [P14408-1]

Organism-specific databases

RGDi2614. Fh.

Phylogenomic databases

eggNOGiKOG1317. Eukaryota.
COG0114. LUCA.
HOGENOMiHOG000061736.
HOVERGENiHBG002183.
InParanoidiP14408.
PhylomeDBiP14408.

Enzyme and pathway databases

UniPathwayiUPA00223; UER01007.
SABIO-RKP14408.

Miscellaneous databases

PROiP14408.

Family and domain databases

Gene3Di1.10.275.10. 1 hit.
HAMAPiMF_00743. FumaraseC. 1 hit.
InterProiIPR005677. Fum_hydII.
IPR024083. Fumarase/histidase_N.
IPR018951. Fumarase_C_C.
IPR020557. Fumarate_lyase_CS.
IPR000362. Fumarate_lyase_fam.
IPR022761. Fumarate_lyase_N.
IPR008948. L-Aspartase-like.
[Graphical view]
PANTHERiPTHR11444. PTHR11444. 1 hit.
PfamiPF10415. FumaraseC_C. 1 hit.
PF00206. Lyase_1. 1 hit.
[Graphical view]
PRINTSiPR00149. FUMRATELYASE.
SUPFAMiSSF48557. SSF48557. 1 hit.
TIGRFAMsiTIGR00979. fumC_II. 1 hit.
PROSITEiPS00163. FUMARATE_LYASES. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiFUMH_RAT
AccessioniPrimary (citable) accession number: P14408
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: January 1, 1990
Last modified: November 2, 2016
This is version 144 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

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).By similarity

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.