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Electron Transfer Flavoprotein Beta Polypeptide (ETFb)

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ETF-B; MADD; FP585

Electron Transfer Flavoprotein Beta Polypeptide (ETFb)
CCC B642
HGNC 3482
MGI 106098
PubMed 21903359
RGD 1303312
UniProt P38117
Wiki ETFB

Defects in ETFB are a cause of multiple acyl-CoA dehydrogenase deficiency (MADD); also known as glutaricaciduria IIB and glutaric aciduria IIB (GAIIB). Glutaric aciduria II (GAII) is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism.

GAIIB results in the excretion not only of glutaric acid but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. It is seen in its most severe form in infancy, with polycystic and dysplastic kidneys, hypoketotic acidosis and hypoglycemia that can lead to death. The milder form can be characterized by recurrent episodes of lasting lethargy or slowly progressive myopathy.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB642Hu01 Recombinant Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB642Hu01 Polyclonal Antibody to Electron Transfer Flavoprotein Beta Polypeptide (ETFb) WB; IHC; ICC; IP.
MAB642Hu21 Monoclonal Antibody to Electron Transfer Flavoprotein Beta Polypeptide (ETFb) WB; IHC; ICC; IP.
Assay Kits SEB642Hu ELISA Kit for Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Electron Transfer Flavoprotein Beta Polypeptide (ETFb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Electron Transfer Flavoprotein Beta Polypeptide (ETFb) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Electron Transfer Flavoprotein Beta Polypeptide (ETFb) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Electron Transfer Flavoprotein Beta Polypeptide (ETFb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Electron Transfer Flavoprotein Beta Polypeptide (ETFb) ELISA Kit Customized Service Offer
  1. "cDNA cloning and mitochondrial import of the beta-subunit of the human electron-transfer flavoprotein."Eur. J. Biochem. 213:1003-1008(1993) [PubMed] [Europe PMC] [Abstract]
  2. "Clear relationship between ETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency."Hum. Mutat. 22:12-23(2003) [PubMed] [Europe PMC] [Abstract]
  3. "Large-scale cDNA transfection screening for genes related to cancer development and progression." Proc. Natl. Acad. Sci. U.S.A. 101:15724-15729(2004) [PubMed] [Europe PMC] [Abstract]
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  6. "Mutations and polymorphisms of the gene encoding the beta-subunit of the electron transfer flavoprotein in three patients with glutaric acidemia type II."Hum. Mol. Genet. 3:429-435(1994) [PubMed] [Europe PMC] [Abstract]
  7. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  8. "Human METTL20 methylates lysine residues adjacent to the recognition loop of the electron transfer flavoprotein in mitochondria."J. Biol. Chem. 289:24640-24651(2014) [PubMed] [Europe PMC] [Abstract]
  9. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  10. "Three-dimensional structure of human electron transfer flavoprotein to 2.1-A resolution."Proc. Natl. Acad. Sci. U.S.A. 93:14355-14360(1996) [PubMed] [Europe PMC] [Abstract]
  11. "Extensive domain motion and electron transfer in the human electron transferring flavoprotein.medium chain acyl-CoA dehydrogenase complex."J. Biol. Chem. 279:32904-32912(2004) [PubMed] [Europe PMC] [Abstract]
  12. "Stabilization of non-productive conformations underpins rapid electron transfer to electron-transferring flavoprotein."J. Biol. Chem. 280:30361-30366(2005) [PubMed] [Europe PMC] [Abstract]
  13. "A polymorphic variant in the human electron transfer flavoprotein alpha-chain (alpha-T171) displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with mild childhood presentation."Mol. Genet. Metab. 67:138-147(1999) [PubMed] [Europe PMC] [Abstract]