ACAD8 (GeneName), Isobutyryl-CoA dehydrogenase, mitochondrial (ProteinName), ACAD8_HUMAN.
Product Name:
Human ACAD8/ Isobutyryl-CoA dehydrogenase, mitochondrial ELISA Kit
Cat.#:
E2437h
Brand:
EIAab®
Regulatory Status:
Alternative:
Activator-recruited cofactor 42 kDa component, ARC42, Acyl-CoA dehydrogenase family member 8, ACAD-8, ARC42, IBD, IBDH
Detection Method:
ELISA
Specificity:
Natural and recombinant human Isobutyryl-CoA dehydrogenase, mitochondrial
Sample Type:
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Sample Data:
Research Area:
-
- Data
- Citations
- Publication
- Sequence / 3D
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General Annotation
Sub Unit:
Homotetramer, formed by a dimer of dimers (PubMed:11013134, PubMed:14752098). May be part of the large multiprotein complex ARC/DRIP (PubMed:10235267).
Function:
Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway (PubMed:11013134, PubMed:12359132, PubMed:16857760). To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA (PubMed:11013134, PubMed:12359132).
Subcellular Location:
Mitochondrion
Database link
Valid Sequence:
This product has not yet been referenced specifically in any publications.
[1].
"Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans."
[2].
"Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases."
[3].
"Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism."
[4].
"Isolation and characterisation of a cDNA encoding the precursor for a novel member of the acyl-CoA dehydrogenase gene family."
[5].
"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression."
[7].
"Variations in IBD (ACAD8) in children with elevated C4-carnitine detected by tandem mass spectrometry newborn screening."
[9].
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
[10].
"Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants."
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