Mouse Monoclonal Antibody to ALDH6A1

Supplier: ProMab Technologies
Type of Product: Monoclonal Antibody
Description: 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.;
Application: ELISA: 1/10000; WB: 1/500 - 1/2000; IHC: 1/200 - 1/1000; ICC: 1/200 - 1/1000; FCM: 1/200 - 1/400
Size: 100 ul, 1mg/ml
Species Reactivity: Human
Clone: 6H9B8;
Isotype: Mouse IgG1
Immunogen: Purified recombinant fragment of human ALDH6A1 (AA: 1-195) expressed in E. Coli.
Formulation: Purified antibody in PBS with 0.05% sodium azide
MW: 57.8kDa
Storage: 4C; -20C for long term storage
Supplier link: http://www.promab.com/index.php?main_page=product_info&products_id=4123
Reference: 1.Orphanet J Rare Dis. 2013 Jul 9;8:98. ; 2.J Inherit Metab Dis. 2012 May;35(3):437-42.;

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