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FH

Name fumarate hydratase
Structure PDB 3E04
Interactors 18 interactors: CCDC102B CCDC57 CCNDBP1 CSTPP1 DGCR6 EXOSC5 GRB2 H2AZ1 KLHL6 LMNB2 MDH2 OGT PIM1 POLR1C PRKDC SAT1 TNPO2 TTC19
GO Annotations Cellular Component
  • Nucleus
  • Chromosome
  • Cytoplasm
  • Mitochondrion
  • Mitochondrial Matrix
  • Cytosol
  • Site Of Double-strand Break
  • Extracellular Exosome
Molecular Function
  • Catalytic Activity
  • Fumarate Hydratase Activity
  • Protein Binding
  • Lyase Activity
  • Histone Binding
Biological Process
  • Urea Cycle
  • Regulation Of Arginine Metabolic Process
  • Tricarboxylic Acid Cycle
  • Fumarate Metabolic Process
  • Malate Metabolic Process
  • DNA Repair
  • Arginine Metabolic Process
  • DNA Damage Response
  • Homeostasis Of Number Of Cells Within A Tissue
  • Positive Regulation Of Cold-induced Thermogenesis
  • Positive Regulation Of Double-strand Break Repair Via Nonhomologous End Joining
Pathways
  • Citric acid cycle (TCA cycle)
  • Mitochondrial protein degradation
Diseases
  • Diseases of the tricarboxylic acid cycle, including: Fumarase (FH) deficiency; Succinate dehydrogenase (SDH) deficiency; Alpha-ketoglutarate dehydrogenase (AKGDH) deficiency
  • Multiple cutaneous and uterine leiomyomata
  • Renal cell carcinoma
GWAS
  • Daytime sleep phenotypes ( 27126917 )
  • Major depressive disorder ( 27479909 )
  • Metabolite levels ( 23823483 )
Entrez ID 2271
HPRD ID 00652
Ensembl ID ENSG00000091483
Uniprot IDs A0A0S2Z4C3 P07954
PDB IDs 3E04 5D6B 5UPP 6EBT 6V8F 6VBE 7LUB
GO Terms Enriched among Interactors ?
  • Non-canonical Inflammasome Complex Assembly

© Madhavi K. Ganapathiraju 2012-2026