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Tbio
FH
Fumarate hydratase, mitochondrial

Protein Classes
Protein Summary
Description
Also acts as a tumor suppressor. The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000366560
  • ENSP00000355518
  • ENSG00000091483

Symbol
  • MCL
  • FMRD
  • LRCC
  • HLRCC
  • MCUL1
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
disease perturbation
0.96
kinase perturbation
0.95
protein domain
0.92
co-expressed gene
0.72
biological term
0.71


Related Tools
GENEVA
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GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
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GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 178.07   (req: < 5)
Gene RIFs: 79   (req: <= 3)
Antibodies: 572   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 178.07   (req: >= 5)
Gene RIFs: 79   (req: > 3)
Antibodies: 572   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 6
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
Orthologs (14)
1 – 5 of 14
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
fumarate hydratase
VGNC:10637
457851
Macaque
fumarate hydratase
707542
Mouse
MGI:95530
14194
Rat
RGD:2614
24368
Dog
fumarate hydratase
VGNC:40867
480092
Species
Name
OMA
EggNOG
Inparanoid
Chimp
fumarate hydratase
Macaque
fumarate hydratase
Mouse
Rat
Dog
fumarate hydratase
Protein Structure (3 Structures, 1 AlphaFold Model)
RepresentationColor Scheme

Click on a row in the table to change the structure displayed.
More information can be found at RCSB PDB

AF-P07954-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

1 – 3 of 3
PDB Structure Id
Ligand
Method
Resolution (Å)
Residues
Fraction of Total Protein
Pub Year
Title
PDB Structure Id
Fraction of Total Protein
Resolution
Pub Year
Pathways (23)
Citric acid cycle (TCA cycle) (R-HSA-71403)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 4 of 4
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Citric acid cycle (TCA cycle)
Reactome
Metabolism
Reactome
Pyruvate metabolism and Citric Acid (TCA) cycle
Reactome
The citric acid (TCA) cycle and respiratory electron transport
Name
Explore in Pharos
Explore in Source
Citric acid cycle (TCA cycle)
Metabolism
Pyruvate metabolism and Citric Acid (TCA) cycle
The citric acid (TCA) cycle and respiratory electron transport
Gene Ontology Terms (11)
Items per page:
10
1 – 1 of 1
GO Term
Evidence
Assigned by
Inferred from Experiment (EXP)
Reactome
Protein-Protein Interactions (259)
1 – 10 of 259
MDH2
Tchem
Family:  Enzyme
Novelty:  0.00217694
Score:  0.997
Data Source:  STRINGDB
CS
Tbio
Family:  Enzyme
Novelty:  0.00154214
Score:  0.994
Data Source:  STRINGDB
SDHC
Tbio
Family:  Enzyme
Novelty:  0.0005776
Score:  0.991
Data Source:  STRINGDB
SDHB
Tchem
Family:  Enzyme
Novelty:  0.0010199
Score:  0.99
Data Source:  STRINGDB
SDHD
Tbio
Family:  Enzyme
Novelty:  0.00167904
Score:  0.989
Data Source:  STRINGDB
SDHA
Tbio
Family:  Enzyme
Novelty:  0.00223171
Score:  0.987
Data Source:  STRINGDB
MDH1
Tchem
Family:  Enzyme
Novelty:  0.00203876
Score:  0.983
Data Source:  STRINGDB
ME1
Tchem
Novelty:  0.00553719
Score:  0.967
Data Source:  STRINGDB
OGDH
Tbio
Family:  Enzyme
Novelty:  0.00674601
Score:  0.963
Data Source:  STRINGDB
ME2
Tbio
Novelty:  0.03635548
Score:  0.962
Data Source:  STRINGDB
Publication Statistics
PubMed Score  178.07

PubMed score by year
PubTator Score  487.2

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer
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