ETFDH
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Official Full Name
electron-transferring-flavoprotein dehydrogenase -
Overview
Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane.The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane.Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type IIglutaricacidemia. -
Synonyms
ETFDH;electron-transferring-flavoprotein dehydrogenase;electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial;ETFQO;ETF-QO;ETF dehydrogenase;ETF-ubiquinone oxidoreductase;electron transfer flavoprotein ubiquinone oxidoreductase;MA
Recombinant Proteins
- Zebrafish
- Chicken
- Human
- Mouse
- Pig
- Rat
- Mammalian Cell
- Wheat Germ
- HEK293
- E.coli
- Yeast
- In Vitro Cell Free System
- His
- GST
- Non
- His&SUMO
- His&Fc&Avi
Involved Pathway
ETFDH involved in several pathways and played different roles in them. We selected most pathways ETFDH participated on our site, such as Metabolism,Respiratory electron transport,Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins., which may be useful for your reference. Also, other proteins which involved in the same pathway with ETFDH were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Respiratory electron transport | ETFB,COX14,TACO1,FAM36A,NDUFAF3,COX11,NDUFAF6,NDUFAF5,SCO1,TIMMDC1 |
| Metabolism | DECR1,SLC25A1,YY2,ABHD14B,CYP2F1,NAMPT,GLYAT,OSBP,SPRB,AGPAT6 |
| The citric acid (TCA) cycle and respiratory electron transport | C2orf56,PDK2,COX16,UCP2,SLC25A27,PDK3,ADHFE1,SLC25A14,ETFB,PDP2 |
| Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. | NDUFAF5,COX11,NDUFAF4,COX18,SLC25A27,COX14,UCP2,SCO1,ATP5L,NDUFAF3 |
Protein Function
ETFDH has several biochemical functions, for example, 4 iron, 4 sulfur cluster binding,electron carrier activity,electron-transferring-flavoprotein dehydrogenase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by ETFDH itself. We selected most functions ETFDH had, and list some proteins which have the same functions with ETFDH. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| metal ion binding | TP63,IGFBP3,ZKSCAN1,ARHGEF1A,ATP13A3,ADAL,PHYH,ZNF502,IRF2BPL,ZFP474 |
| oxidoreductase activity | CYP3C1L2,SARDH,CYP2C54,SDR39U1,DAO.3,GPD1B,CYP4A12A,SDR16C5B,CYP2K19,CYP2P10 |
| flavin adenine dinucleotide binding | DUS1L,DUS3L,NOS1,ETFA,DLD,NOS3,SDHA,KDM1A,GCDH,POR |
| 4 iron, 4 sulfur cluster binding | RSAD2,MOCS1,NUBP2,NDUFS8,POLA1,ISCUB,POLD1,NDUFS8A,RSAD1,ERCC2 |
| electron-transferring-flavoprotein dehydrogenase activity | AIFM2 |
| electron carrier activity | QDPR,NQO2,GCDHB,ACAD11,ETFA,NCF1,GLRX2,TXNRD3,GRX2,AOX1 |
| quinone binding | VKORC1L1,HHIPL1,HHIP,AOC1,NDUFS7,AOC3,VKORC1,TP53I3,ABP1,HHIPL2 |
| ubiquinone binding | SDHDA,SDHD,DHODH,SDHB,SDHDB |
Interacting Protein
ETFDH 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 ETFDH here. Most of them are supplied by our site. Hope this information will be useful for your research of ETFDH.
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References
- Shioya, A; Takuma, H; et al. Amelioration of acylcarnitine profile using bezafibrate and riboflavin in a case of adult-onset glutaric acidemia type 2 with novel mutations of the electron transfer flavoprotein dehydrogenase (ETFDH) gene. JOURNAL OF THE NEUROLOGICAL SCIENCES 346:350-352(2014).
- Cornelius, N; Corydon, TJ; et al. Cellular consequences of oxidative stress in riboflavin responsive multiple acyl-CoA dehydrogenation deficiency patient fibroblasts. HUMAN MOLECULAR GENETICS 23:4285-4301(2014).
