UniProtKB - Q7SX99 (FUMH_DANRE)
Fumarate hydratase, mitochondrial
fh
Functioni
Catalyzes the reversible stereospecific interconversion of fumarate to L-malate (By similarity).
Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable).
By similarityCuratedCatalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH.
By similarityCatalyzes the dehydration of L-malate to fumarate. Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism (By similarity).
Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ). In response to DNA damage translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate (By similarity).
By similarityMiscellaneous
Catalytic activityi
- EC:4.2.1.2By similarity EC:4.2.1.2By similarityThis reaction proceeds in the backwardBy similarity direction.
: tricarboxylic acid cycle Pathwayi
This protein is involved in step 1 of the subpathway that synthesizes (S)-malate from fumarate.By similarity 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 key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 233 | SubstrateBy similarity | 1 | |
Active sitei | 234 | Proton donor/acceptorBy similarity | 1 | |
Active sitei | 364 | By similarity | 1 | |
Binding sitei | 365 | SubstrateBy similarity | 1 | |
Sitei | 377 | Important for catalytic activityBy similarity | 1 |
GO - Molecular functioni
- fumarate hydratase activity Source: UniProtKB
GO - Biological processi
- cellular response to DNA damage stimulus Source: UniProtKB
- DNA repair Source: UniProtKB-KW
- fumarate metabolic process Source: UniProtKB
- malate metabolic process Source: UniProtKB
- negative regulation of histone H3-K36 methylation Source: UniProtKB
- positive regulation of double-strand break repair via nonhomologous end joining Source: UniProtKB
- regulation of arginine metabolic process Source: UniProtKB
- tricarboxylic acid cycle Source: GO_Central
- urea cycle Source: UniProtKB
Keywordsi
Molecular function | Lyase |
Biological process | DNA damage, DNA repair, Tricarboxylic acid cycle |
Enzyme and pathway databases
Reactomei | R-DRE-71403, Citric acid cycle (TCA cycle) |
UniPathwayi | UPA00223;UER01007 |
Names & Taxonomyi
Protein namesi | Recommended name: Fumarate hydratase, mitochondrialBy similarity (EC:4.2.1.2By similarity)Short name: FumaraseBy similarity |
Gene namesi | Name:fhBy similarity ORF Names:zgc:662531 Publication |
Organismi | Danio rerio (Zebrafish) (Brachydanio rerio) |
Taxonomic identifieri | 7955 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Actinopterygii › Neopterygii › Teleostei › Ostariophysi › Cypriniformes › Danionidae › Danioninae › Danio |
Proteomesi |
|
Organism-specific databases
ZFINi | ZDB-GENE-010724-6, fh |
Subcellular locationi
Mitochondrion
- Mitochondrion By similarity
Nucleus
- Nucleus By similarity
Cytoplasm and Cytosol
- cytosol By similarity
Other locations
- Chromosome By similarity
Note: Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs).By similarity
Cytosol
- cytosol Source: UniProtKB-SubCell
Mitochondrion
- mitochondrion Source: GO_Central
Nucleus
- nucleus Source: UniProtKB-SubCell
Other locations
- chromosome Source: UniProtKB-SubCell
- tricarboxylic acid cycle enzyme complex Source: InterPro
Keywords - Cellular componenti
Chromosome, Cytoplasm, Mitochondrion, NucleusPTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length | |
---|---|---|---|---|---|
Transit peptidei | 1 – 43 | MitochondrionBy similarityAdd BLAST | 43 | ||
ChainiPRO_0000010327 | 44 – 509 | Fumarate hydratase, mitochondrialAdd BLAST | 466 | ||
Isoform Cytoplasmic (identifier: Q7SX99-2) | |||||
Initiator methioninei | RemovedBy similarity |
Proteomic databases
PaxDbi | Q7SX99 |
Interactioni
Subunit structurei
Homotetramer.
By similarityProtein-protein interaction databases
STRINGi | 7955.ENSDARP00000097494 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 144 – 146 | Substrate bindingBy similarity | 3 | |
Regioni | 175 – 178 | Substrate binding (B site)By similarity | 4 | |
Regioni | 185 – 187 | Substrate bindingBy similarity | 3 | |
Regioni | 370 – 372 | Substrate bindingBy similarity | 3 |
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG1317, Eukaryota |
InParanoidi | Q7SX99 |
OrthoDBi | 1022919at2759 |
PhylomeDBi | Q7SX99 |
Family and domain databases
CDDi | cd01362, Fumarase_classII, 1 hit |
Gene3Di | 1.10.275.10, 1 hit |
HAMAPi | MF_00743, FumaraseC, 1 hit |
InterProi | View 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 |
PANTHERi | PTHR11444, PTHR11444, 1 hit |
Pfami | View protein in Pfam PF10415, FumaraseC_C, 1 hit PF00206, Lyase_1, 1 hit |
PRINTSi | PR00149, FUMRATELYASE |
SUPFAMi | SSF48557, SSF48557, 1 hit |
TIGRFAMsi | TIGR00979, fumC_II, 1 hit |
PROSITEi | View protein in PROSITE PS00163, FUMARATE_LYASES, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative initiation. AlignAdd to basketThis entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MYRSARSLHR FSASLSDLRA AQRSIKARNV CPAPGLRHQT VRMASSEAFR
60 70 80 90 100
IERDTFGELK VPSDKYYGAQ TVRSTMNFRI GGVTERMPIQ VIRAFGILKK
110 120 130 140 150
AAAEVNKDYG LDPKIADAIM KAADEVESGK LDDHFPLVVW QTGSGTQTNM
160 170 180 190 200
NVNEVISNRA IEMLGGKLGS KDPVHPNDHV NKSQSSNDTF PTAMHIAAAK
210 220 230 240 250
EVHEVLLPGL QTLHDALAAK AEQFKDIIKI GRTHTQDAVP LSLGQEFGGY
260 270 280 290 300
VQQVKYSIAR VKASLPRVYE LAAGGTAVGT GLNTRIGFAE KVADKVSALT
310 320 330 340 350
GLPFVTAANK FEALAAHDAL VELSGALNTV AVSMMKIAND IRFLGSGPRS
360 370 380 390 400
GLGELILPEN EPGSSIMPGK VNPTQCEAMT MVAAQVMGNH VAVTVGGSNG
410 420 430 440 450
HFELNVFKPM IIKNVLNSAR LLGDASVSFT NNCVVGIEAN TERINKLMSE
460 470 480 490 500
SLMLVTALNP HIGYDKAAKI AKTAHKDGST LKEAALKLGF LNEQQFEEWV
RPHDMLGPK
The sequence of this isoform differs from the canonical sequence as follows:
1-42: Missing.
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketF1QWR4 | F1QWR4_DANRE | Fumarate hydratase, mitochondrial | fh | 509 | Annotation score: |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_060137 | 1 – 42 | Missing in isoform Cytoplasmic. Add BLAST | 42 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | BC055566 mRNA Translation: AAH55566.1 BC066484 mRNA Translation: AAH66484.1 |
RefSeqi | NP_957257.1, NM_200963.1 [Q7SX99-1] |
Genome annotation databases
GeneIDi | 393938 |
KEGGi | dre:393938 |
Keywords - Coding sequence diversityi
Alternative initiationSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | BC055566 mRNA Translation: AAH55566.1 BC066484 mRNA Translation: AAH66484.1 |
RefSeqi | NP_957257.1, NM_200963.1 [Q7SX99-1] |
3D structure databases
SMRi | Q7SX99 |
ModBasei | Search... |
Protein-protein interaction databases
STRINGi | 7955.ENSDARP00000097494 |
Proteomic databases
PaxDbi | Q7SX99 |
Genome annotation databases
GeneIDi | 393938 |
KEGGi | dre:393938 |
Organism-specific databases
CTDi | 2271 |
ZFINi | ZDB-GENE-010724-6, fh |
Phylogenomic databases
eggNOGi | KOG1317, Eukaryota |
InParanoidi | Q7SX99 |
OrthoDBi | 1022919at2759 |
PhylomeDBi | Q7SX99 |
Enzyme and pathway databases
UniPathwayi | UPA00223;UER01007 |
Reactomei | R-DRE-71403, Citric acid cycle (TCA cycle) |
Miscellaneous databases
PROi | PR:Q7SX99 |
Family and domain databases
CDDi | cd01362, Fumarase_classII, 1 hit |
Gene3Di | 1.10.275.10, 1 hit |
HAMAPi | MF_00743, FumaraseC, 1 hit |
InterProi | View 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 |
PANTHERi | PTHR11444, PTHR11444, 1 hit |
Pfami | View protein in Pfam PF10415, FumaraseC_C, 1 hit PF00206, Lyase_1, 1 hit |
PRINTSi | PR00149, FUMRATELYASE |
SUPFAMi | SSF48557, SSF48557, 1 hit |
TIGRFAMsi | TIGR00979, fumC_II, 1 hit |
PROSITEi | View protein in PROSITE PS00163, FUMARATE_LYASES, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | FUMH_DANRE | |
Accessioni | Q7SX99Primary (citable) accession number: Q7SX99 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | December 15, 2003 |
Last sequence update: | October 1, 2003 | |
Last modified: | December 2, 2020 | |
This is version 106 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- PATHWAY comments
Index of metabolic and biosynthesis pathways - SIMILARITY comments
Index of protein domains and families