ACADSB (基因名), Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial (蛋白名), ACDSB_HUMAN.
Human ACADSB/ Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial ELISA Kit
SBCAD, 2-methyl branched chain acyl-CoA dehydrogenase, 2-MEBCAD, 2-methylbutyryl-coenzyme A dehydrogenase, 2-methylbutyryl-CoA dehydrogenase
Natural and recombinant human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
MEGLAVRLLR | GSRLLRRNFL | TCLSSWKIPP | HVSKSSQSEA | LLNITNNGIH |
FAPLQTFTDE | EMMIKSSVKK | FAQEQIAPLV | STMDENSKME | KSVIQGLFQQ |
GLMGIEVDPE | YGGTGASFLS | TVLVIEELAK | VDASVAVFCE | IQNTLINTLI |
RKHGTEEQKA | TYLPQLTTEK | VGSFCLSEAG | AGSDSFALKT | RADKEGDYYV |
LNGSKMWISS | AEHAGLFLVM | ANVDPTIGYK | GITSFLVDRD | TPGLHIGKPE |
NKLGLRASST | CPLTFENVKV | PEANILGQIG | HGYKYAIGSL | NEGRIGIAAQ |
MLGLAQGCFD | YTIPYIKERI | QFGKRLFDFQ | GLQHQVAHVA | TQLEAARLLT |
YNAARLLEAG | KPFIKEASMA | KYYASEIAGQ | TTSKCIEWMG | GVGYTKDYPV |
EKYFRDAKIG | TIYEGASNIQ | LNTIAKHIDA | EY
"Short/branched-chain acyl-CoA dehydrogenase deficiency due to an IVS3+3A>G mutation that causes exon skipping."
"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."
"Localization of short/branched chain acyl-CoA dehydrogenase (ACADSB) to human chromosome 10."
"Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family."
"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression."
"Association of genetic polymorphisms of ACADSB and COMT with human hypertension."
"A scan of chromosome 10 identifies a novel locus showing strong association with late-onset Alzheimer disease."
"Inborn errors of isoleucine degradation: a review."
"2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation."
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
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