ACAD8 (GeneName), Isobutyryl-CoA dehydrogenase, mitochondrial (ProteinName), ACAD8_HUMAN.
Human ACAD8/ Isobutyryl-CoA dehydrogenase, mitochondrial ELISA Kit
Activator-recruited cofactor 42 kDa component, ARC42, Acyl-CoA dehydrogenase family member 8, ACAD-8, ARC42, IBD, IBDH
Natural and recombinant human Isobutyryl-CoA dehydrogenase, mitochondrial
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Homotetramer, formed by a dimer of dimers (PubMed:11013134, PubMed:14752098). May be part of the large multiprotein complex ARC/DRIP (PubMed:10235267).
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).
MLWSGCRRFG | ARLGCLPGGL | RVLVQTGHRS | LTSCIDPSMG | LNEEQKEFQK |
VAFDFAAREM | APNMAEWDQ | KELFPVDVMR | KAAQLGFGGV | YIQTDVGGSG |
LSRLDTSVIF | EALATGCTST | T AYISIHNM | CAWMIDSFGN | EEQRHKFCPP |
LCTMEKFASY | CLTEPGSGSD | AASLLTSAKK | QG DHYILNG | SKAFISGAGE |
SDIYVVMCRT | GGPGPKGISC | IVVEKGTPGL | SFGKKEKKVG | WNS QPTRAV |
IFEDCAVPVA | NRIGSEGQGF | LIAVRGLNGG | RINIASCSLG | AAHASVILTR |
DHLN VRKQF | GEPLASNQYL | QFTLADMATR | LVAARLMVRN | AAVALQEERK |
DAVALCSMAK | LFATD ECFA | ICNQALQMHG | GYGYLKDYAV | QQYVRDSRVH |
QILEGSNEVM | RILISRSLLQ | E
This product has not yet been referenced specifically in any publications.
"Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans."
"Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases."
"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."
"Isolation and characterisation of a cDNA encoding the precursor for a novel member of the acyl-CoA dehydrogenase gene family."
"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression."
"Association study of cholesterol-related genes in Alzheimer's disease."
"Variations in IBD (ACAD8) in children with elevated C4-carnitine detected by tandem mass spectrometry newborn screening."
"Complete sequencing and characterization of 21,243 full-length human cDNAs."
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
"Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants."
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