ALDH6A1 Antibody
Purified Mouse Monoclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| WB, IHC, FC, ICC, E |
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Primary Accession | Q02252 |
Reactivity | Human |
Host | Mouse |
Clonality | Monoclonal |
Clone Names | 6H9B8 |
Isotype | IgG1 |
Calculated MW | 57.8kDa |
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 |
Gene ID | 4329 |
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Other Names | Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial, MMSDH, Malonate-semialdehyde dehydrogenase [acylating], 1.2.1.18, 1.2.1.27, Aldehyde dehydrogenase family 6 member A1, ALDH6A1, MMSDH |
Dilution | E~~1/10000 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. |
Name | ALDH6A1 (HGNC:7179) |
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Function | Malonate and methylmalonate semialdehyde dehydrogenase involved in the catabolism of valine, thymine, and compounds catabolized by way of beta-alanine, including uracil and cytidine. |
Cellular Location | Mitochondrion. |
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Provided below are standard protocols that you may find useful for product applications.
References
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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