|Application ||WB, IHC, FC, ICC, E|
|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.|
|Immunogen||Purified recombinant fragment of human ALDH6A1 (AA: 1-195) expressed in E. Coli.|
|Formulation||Purified antibody in PBS with 0.05% sodium azide|
|Other Names||Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial, MMSDH, Malonate-semialdehyde dehydrogenase [acylating], 22.214.171.124, 126.96.36.199, Aldehyde dehydrogenase family 6 member A1, ALDH6A1, MMSDH|
WB~~1/500 - 1/2000
IF~~1/200 - 1/1000
FC~~1/200 - 1/400
IHC~~1/200 - 1/1000
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||ALDH6A1 Antibody 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.|
Thousands of laboratories across the world have published research that depended on the performance of antibodies from Abgent to advance their research. Check out links to articles that cite our products in major peer-reviewed journals, organized by research category.
firstname.lastname@example.org, and receive a free "I Love Antibodies" mug.
Provided below are standard protocols that you may find useful for product applications.
1.Orphanet J Rare Dis. 2013 Jul 9;8:98.2.J Inherit Metab Dis. 2012 May;35(3):437-42.
If you have used an Abgent product and would like to share how it has performed, please click on the "Submit Review" button and provide the requested information. Our staff will examine and post your review and contact you if needed.
If you have any additional inquiries please email technical services at email@example.com.