|Application ||IHC-P, WB, E|
|Calculated MW||57840 Da|
|Antigen Region||367-396 aa|
|Other Names||Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial, MMSDH, Malonate-semialdehyde dehydrogenase [acylating], Aldehyde dehydrogenase family 6 member A1, ALDH6A1, MMSDH|
|Target/Specificity||This ALDH6A1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 367-396 amino acids from the C-terminal region of human ALDH6A1.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||ALDH6A1 Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.|
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Provided below are standard protocols that you may find useful for product applications.
ALDH6A1 belongs to the aldehyde dehydrogenases family of proteins. This enzyme plays a role in the valine and pyrimidine catabolic pathways. This protein is a mitochondrial methylmalonate semialdehyde dehydrogenase, and 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.
Kuiper,H., Cytogenet. Genome Res. 109 (4), 533 (2005)
Anderson,N.L., Mol. Cell Proteomics 3 (4), 311-326 (2004)
Chambliss,K.L., J. Inherit. Metab. Dis. 23 (5), 497-504 (2000)
Kedishvili,N.Y., J. Biol. Chem. 267 (27), 19724-19729 (1992)
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