Cell Biology Antibodies 10

Anti-ETFDH Antibody (CAB6585)

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SKU:
CAB6585
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-ETFDH Antibody
Antibody SKU:CAB6585
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 318-617 of human ETFDH (NP_004444.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:Mouse liver, Mouse heart, Mouse kidney, Rat liver, Rat heart
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 318-617 of human ETFDH (NP_004444.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:LVAL GLVV GLDY QNPY LSPF REFQ RWKH HPSI RPTL EGGK RIAY GARA LNEG GFQS IPKL TFPG GLLI GCSP GFMN VPKI KGTH TAMK SGIL AAES IFNQ LTSE NLQS KTIG LHVT EYED NLKN SWVW KELY SVRN IRPS CHGV LGVY GGMI YTGI FYWI LRGM EPWT LKHK GSDF ERLK PAKD CTPI EYPK PDGQ ISFD LLSS VALS GTNH EHDQ PAHL TLRD DSIP VNRN LSIY DGPE QRFC PAGV YEFV PVEQ GDGF RLQI NAQN CVHC KTCD IKDP SQNI NWVV PEGG GGPA YNGM
Gene ID:2110
Uniprot:Q16134
Cellular Location:Mitochondrion inner membrane
Calculated MW:62kDa/68kDa
Observed MW:65kDa
Synonyms:ETFDH, ETFQO, MADD
Background:This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed.
UniProt Protein Function:ETFDH: Accepts electrons from ETF and reduces ubiquinone. Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C). GA2C is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Belongs to the ETF-QO/FixC family.
UniProt Protein Details:

Protein type:Membrane protein, integral; Mitochondrial; Oxidoreductase; EC 1.5.5.1

Chromosomal Location of Human Ortholog: 4q32-q35

Cellular Component: mitochondrial matrix; mitochondrial membrane; integral to mitochondrial inner membrane

Molecular Function:ubiquinone binding; FAD binding; electron carrier activity; metal ion binding; 4 iron, 4 sulfur cluster binding; oxidoreductase activity, oxidizing metal ions with flavin as acceptor; oxidoreductase activity; quinone binding; electron-transferring-flavoprotein dehydrogenase activity

Biological Process: cellular metabolic process; response to oxidative stress; fatty acid beta-oxidation using acyl-CoA dehydrogenase

Disease: Multiple Acyl-coa Dehydrogenase Deficiency

NCBI Summary:This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed. [provided by RefSeq, Aug 2013]
UniProt Code:Q16134
NCBI GenInfo Identifier:292495008
NCBI Gene ID:2110
NCBI Accession:Q16134.2
UniProt Secondary Accession:Q16134,Q7Z347, B4E3R9, J3KND9,
UniProt Related Accession:Q16134,AAB24227
Molecular Weight:68,495 Da
NCBI Full Name:Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
NCBI Synonym Full Names:electron-transferring-flavoprotein dehydrogenase
NCBI Official Symbol:ETFDH  
NCBI Official Synonym Symbols:MADD; ETFQO  
NCBI Protein Information:electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; ETF-QO; ETF dehydrogenase; ETF-ubiquinone oxidoreductase
UniProt Protein Name:Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial
UniProt Synonym Protein Names:Electron-transferring-flavoprotein dehydrogenase
UniProt Gene Name:ETFDH  
UniProt Entry Name:ETFD_HUMAN
Anti-ETFDH Antibody (CAB6585)Western blot analysis of extracts of various cell lines, using ETFDH antibody (CAB6585) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 3s.
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