This gene encodes a member of the aldehyde dehydrogenase protein family. The encoded protein is a mitochondrial methylmalonate semialdehyde dehydrogenase that plays a role in the valine and pyrimidine catabolic pathways. This protein catalyzes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids. Alternate splicing results in multiple transcript variants.
应用类型
IHC-P, WB
免疫原
Recombinant protein encompassing a sequence within the center region of human ALDH6A1. The exact sequence is proprietary.
来源宿主
Rabbit
反应性
该ALDH6A1 抗体的反应性请参考该产品的说明书
保存建议
Keep as concentrated solution. Aliquot and store at -20C or below. Avoid multiple freeze-thaw cycles.
其他
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