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Protein

Fumarate hydratase, mitochondrial

Gene

Fh

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

Functioni

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

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

Keywordsi

Molecular functionLyase
Biological processTricarboxylic acid cycle

Enzyme and pathway databases

SABIO-RKiP14408
UniPathwayi
UPA00223;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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi2614 Fh

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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
SMRiP14408
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

CDDicd01362 Fumarase_classII, 1 hit
Gene3Di1.10.275.10, 1 hit
HAMAPiMF_00743 FumaraseC, 1 hit
InterProiView protein in InterPro
IPR005677 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
PANTHERiPTHR11444 PTHR11444, 1 hit
PfamiView protein in Pfam
PF10415 FumaraseC_C, 1 hit
PF00206 Lyase_1, 1 hit
PRINTSiPR00149 FUMRATELYASE
SUPFAMiSSF48557 SSF48557, 1 hit
TIGRFAMsiTIGR00979 fumC_II, 1 hit
PROSITEiView protein in PROSITE
PS00163 FUMARATE_LYASES, 1 hit

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

This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q5M964Q5M964_RAT
Fumarate hydratase 1
Fh Fh1, rCG_20230
507Annotation score:

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

Similar proteinsi

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
SMRiP14408
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

UniPathwayi
UPA00223;UER01007

SABIO-RKiP14408

Miscellaneous databases

PROiPR:P14408

Family and domain databases

CDDicd01362 Fumarase_classII, 1 hit
Gene3Di1.10.275.10, 1 hit
HAMAPiMF_00743 FumaraseC, 1 hit
InterProiView protein in InterPro
IPR005677 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
PANTHERiPTHR11444 PTHR11444, 1 hit
PfamiView protein in Pfam
PF10415 FumaraseC_C, 1 hit
PF00206 Lyase_1, 1 hit
PRINTSiPR00149 FUMRATELYASE
SUPFAMiSSF48557 SSF48557, 1 hit
TIGRFAMsiTIGR00979 fumC_II, 1 hit
PROSITEiView protein in PROSITE
PS00163 FUMARATE_LYASES, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiFUMH_RAT
AccessioniPrimary (citable) accession number: P14408
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: January 1, 1990
Last modified: September 12, 2018
This is version 150 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
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Main funding by: National Institutes of Health

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