FH
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Official Full Name
fumarate hydratase -
Overview
Frataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle (Campuzano, 1996; Koutnikova, 1997). Frataxin is expressed as a 30 kDa precursor (transient; 210 amino acids) that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase (MPP) to yield the mature protein (Koutnikova, 1998). The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20 kDa (aa 42-210). Further cleavage of the N-terminus of this 20 kDa intermediate by MPP results in the mature 18 kDa frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich's ataxia, an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy. In the majority of cases of Friedreich's ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription (Campuzano, 1997; Bidichandani, 1998). This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress (Foury, 1997; for review see Puccio, 2000). -
Synonyms
FH;fumarate hydratase;fumarate hydratase, mitochondrial;fumarase;MCL;LRCC;HLRCC;MCUL1
Recombinant Proteins
- Zebrafish
- Human
- Chicken
- Cynomolgus
- Rat
- Mammalian Cell
- E.coli
- HEK293
- Normal rat serum from healthy animals
- Wheat Germ
- HEK293T
- In Vitro Cell Free System
- His
- Non
- His&T7
- His&Fc&Avi
- GST
- Myc&DDK
Involved Pathway
FH involved in several pathways and played different roles in them. We selected most pathways FH participated on our site, such as Citrate cycle (TCA cycle),Pyruvate metabolism,Metabolic pathways, which may be useful for your reference. Also, other proteins which involved in the same pathway with FH were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Pyruvate metabolism | LDHC,hchA,LDHB,MDH2,FH1,ACYP2,BRP44,PKM,PDK2,PDK3B |
| Renal cell carcinoma | RBX1,ARNT2,TGFB2,EP300,PAK7,MAPK3,PAK3,HRAS,PIK3CG,HGF |
| Pathways in cancer | RXRG,LAMC1,FZD5,WNT2,PLCG2,ITGAV,WNT1,LPAR3,FGF19,TRAF2 |
| Metabolic pathways | NMNAT2,MTMR8,ALG10B,ENO1A,SPTLC3,MGAT4A,DGKE,GUK1,PLA2G4C,RFK |
| Citrate cycle (TCA cycle) | ACO2,SDHB,PCK1,SDHD,OGDH,DLDH,PDHB,SDHC,IDH3B,SDHA |
| Carbon metabolism | DAK,ECHS1,CS,AGXT,ADPGK,SDHDB,PDHA1A,PFKMA,ANI_1_2390104,GPR19 |
Protein Function
FH has several biochemical functions, for example, fumarate hydratase activity,protein binding. Some of the functions are cooperated with other proteins, some of the functions could acted by FH itself. We selected most functions FH had, and list some proteins which have the same functions with FH. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| protein binding | LGI4,DOLK,STARD13,TRPC6,KDRL,HDAC1,TMEM120B,MLLT6,MAP4K2,CAPRIN2 |
| fumarate hydratase activity | FH1 |
Interacting Protein
FH has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with FH here. Most of them are supplied by our site. Hope this information will be useful for your research of FH.
PIM1;fumaric acid;(-)-epigallocatechin 3-gallate;EIF6;IKBKE;PRKAB1;GRB2;asnA;q81z75_bacan;uvrB;ATF2;IQCB1
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References
- Jialal, I; Patel, SB; et al. PCSK9 Inhibitors: The Next Frontier in Low-Density Lipoprotein Lowering. METABOLIC SYNDROME AND RELATED DISORDERS 13:99-101(2015).
- Crotty, S; et al. A brief history of T cell help to B cells. NATURE REVIEWS IMMUNOLOGY 15:185-189(2015).
