ACAD8 (基因名), Isobutyryl-CoA dehydrogenase, mitochondrial (蛋白名), ACAD8_BOVIN.
Bovine ACAD8/ Isobutyryl-CoA dehydrogenase, mitochondrial ELISA Kit
Acyl-CoA dehydrogenase family member 8, ACAD-8
Natural and recombinant bovine Isobutyryl-CoA dehydrogenase, mitochondrial
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Homotetramer, formed by a dimer of dimers. Subunit of the large multiprotein complex ARC/DRIP.
Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism.
MMLRGGCQRV | GARLRGLRRG | PRGPADGARR | GVVSCIDPSM | GLSEEQKEFQ |
KVAFNFAARE | MAPHMAEWDQ | KELFPVDTMR | KAAQLGFGGV | YVQTDVGGAG |
LSRLDTSIIF | EALATGCTST | TAYMSIHNMC | VWIIDRFGSE | EQRHRLCPPL |
CTMEKFASYC | LTEPGSGSDA | ASLMTSAVRQ | HDHYILNGSK | AFISGGGEAD |
IYVVMCRTGG | PGPRGISCVV | VEKGTPGLSF | GKKEKKVGWN | SQPTQAVIFE |
DCAVPVANRI | GDEGQGFLIA | MKGLNGGRIN | VASCSLGAAH | ASIVLARDYL |
KVRKQFGEPL | ANSQYLQFQL | ADMAARLVAS | RLMIRTAATA | LQEEREDAIV |
LCSMAKLFAT | DECFAICNQA | LQMHGGYGYL | KDYAVQQYVR | DSRVHQILEG |
SNEVMRMLIS | RSLLQE
"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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