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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


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: 300.48   (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: 300.48   (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
Protein Data Bank (3)
1 – 3 of 3
PDB Structure Id
Ligand
Method
Resolution (Å)
M.W. (kDa)
Pub Year
Title
PDB Structure Id
M.W.
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: 0.00378295
Score: 0.997
Data Source: STRINGDB
CS
Tbio
Family: Enzyme
Novelty: 0.00182322
Score: 0.994
Data Source: STRINGDB
SDHC
Tbio
Family: Enzyme
Novelty: 0.00173432
Score: 0.991
Data Source: STRINGDB
SDHB
Tbio
Family: Enzyme
Novelty: 0.0020679
Score: 0.99
Data Source: STRINGDB
SDHD
Tbio
Family: Enzyme
Novelty: 0.00222227
Score: 0.989
Data Source: STRINGDB
SDHA
Tbio
Family: Enzyme
Novelty: 0.00476895
Score: 0.987
Data Source: STRINGDB
MDH1
Tbio
Family: Enzyme
Novelty: 0.00241236
Score: 0.983
Data Source: STRINGDB
ME1
Tchem
Novelty: 0.00792221
Score: 0.967
Data Source: STRINGDB
OGDH
Tbio
Family: Enzyme
Novelty: 0.01059143
Score: 0.963
Data Source: STRINGDB
ME2
Tbio
Novelty: 0.01912856
Score: 0.962
Data Source: STRINGDB
Publication Statistics
PubMed Score  300.48

PubMed score by year
PubTator Score  487.2

PubTator score by year
Amino Acid Sequence
Residue Counts
Sequence
MYRALRLLARSRPLVRAPAAALASAPGLGGAAVPSFWPPNAARMASQNSFRIEYDTFGELKVPNDKYYGA
1-70
QTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPLVV
70-140
WQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSNDTFPTAMHIAAAIEVHEVLLPG
140-210
LQKLHDALDAKSKEFAQIIKIGRTHTQDAVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVG
210-280
TGLNTRIGFAEKVAAKVAALTGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRFLGSGPR
280-350
SGLGELILPENEPGSSIMPGKVNPTQCEAMTMVAAQVMGNHVAVTVGGSNGHFELNVFKPMMIKNVLHSA
350-420
RLLGDASVSFTENCVVGIQANTERINKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGSTLKETAIELG
420-490
YLTAEQFDEWVKPKDMLGPK
490-510
MYRALRLLARSRPLVRAPAAALASAPGLGGAAVPSFWPPNAARMASQNSFRIEYDTFGELKVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPLVVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSNDTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQDAVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIGFAEKVAAKVAALTGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRFLGSGPRSGLGELILPENEPGSSIMPGKVNPTQCEAMTMVAAQVMGNHVAVTVGGSNGHFELNVFKPMMIKNVLHSARLLGDASVSFTENCVVGIQANTERINKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGSTLKETAIELGYLTAEQFDEWVKPKDMLGPK