Ferroptosis Regulator Information

General Information of the Ferroptosis Regulator (ID: REG10080)
Regulator Name Fumarate hydratase, mitochondrial (FH)
Gene Name FH
Gene ID 2271
Regulator Type Protein coding
Uniprot ID P07954
Sequence
MYRALRLLARSRPLVRAPAAALASAPGLGGAAVPSFWPPNAARMASQNSFRIEYDTFGEL
KVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQDYGLDPKIANAI
MKAADEVAEGKLNDHFPLVVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDH
VNKSQSSNDTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQDAV
PLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIGFAEKVAAKVAAL
TGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRFLGSGPRSGLGELILPE
NEPGSSIMPGKVNPTQCEAMTMVAAQVMGNHVAVTVGGSNGHFELNVFKPMMIKNVLHSA
RLLGDASVSFTENCVVGIQANTERINKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGS
TLKETAIELGYLTAEQFDEWVKPKDMLGPK

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Family Class-II fumarase/aspartase family
Function
Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable).

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HGNC ID
HGNC:3700
KEGG ID hsa:2271
Full List of the Ferroptosis Target of This Regulator and Corresponding Disease/Drug Response(s)
FH can regulate the following target(s), and cause disease/drug response(s). You can browse detail information of target(s) or disease/drug response(s).
Browse Target
Browse Disease
Phospholipid hydroperoxide glutathione peroxidase (GPX4) [Suppressor]
 Target Info 
In total 1 item(s) under this target
Experiment 1 Reporting the Ferroptosis Target of This Regulator [1]
Target for Ferroptosis Suppressor
Responsed Disease Hereditary Leiomyomatosis ICD-11: 2C90
 Disease Info 
Pathway Response Fatty acid metabolism hsa01212
Ferroptosis hsa04216
Cell Process Cell ferroptosis
Cell proliferation
In Vitro Model
 UOK262 cells Hereditary leiomyomatosis Homo sapiens CVCL_1D72
HK-2 cells Normal Homo sapiens CVCL_0302
HT-1080 cells Fibrosarcoma Homo sapiens CVCL_0317
Response regulation Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome characterized by inactivation of the Krebs cycle enzyme fumarate hydratase (FH). Mechanistically, the FH sensitivity to ferroptosis is attributed to dysfunctional GPX4, the primary cellular defender against ferroptosis.
Hereditary Leiomyomatosis [ICD-11: 2C90]
 Disease Info 
In total 1 item(s) under this disease
Experiment 1 Reporting the Ferroptosis-centered Disease Response [1]
Target Regulator Fumarate hydratase, mitochondrial (FH) Protein coding
 Regulator Info 
Pathway Response Fatty acid metabolism hsa01212
Ferroptosis hsa04216
Cell Process Cell ferroptosis
Cell proliferation
In Vitro Model
 UOK262 cells Hereditary leiomyomatosis Homo sapiens CVCL_1D72
HK-2 cells Normal Homo sapiens CVCL_0302
HT-1080 cells Fibrosarcoma Homo sapiens CVCL_0317
Response regulation Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome characterized by inactivation of the Krebs cycle enzyme fumarate hydratase (FH). Mechanistically, the FH sensitivity to ferroptosis is attributed to dysfunctional GPX4, the primary cellular defender against ferroptosis.
References
Ref 1 Fumarate hydratase inactivation in hereditary leiomyomatosis and renal cell cancer is synthetic lethal with ferroptosis induction. Cancer Sci. 2018 Sep;109(9):2757-2766. doi: 10.1111/cas.13701. Epub 2018 Jul 20.