Methylmalonate-Semialdehyde Dehydrogenase [acylating], Mitochondrial (ALDH6A1) Antibody
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Description
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.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Methylmalonate-Semialdehyde Dehydrogenase [acylating], Mitochondrial (ALDH6A1) |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | WB: 1/1000, IHC-P: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified through a protein A column, followed by peptide affinity purification. |
| Size 1 | 80 µl |
| Size 2 | 400 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IHC |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q02252 |
| Alias | ALDH6A1,MMSDH |
| Background | Antibody anti-ALDH6A1 |
| Status | RUO |
Descripción
ALDH6A1, also known as methylmalonate semialdehyde dehydrogenase (MMSDH), is a mitochondrial enzyme involved in the catabolism of branched-chain amino acids (valine and thymine) and odd-chain fatty acids. It catalyzes the oxidation of methylmalonate semialdehyde to propionyl-CoA, which is further metabolized into succinyl-CoA, linking amino acid breakdown to the TCA cycle. ALDH6A1 plays a critical role in energy metabolism and maintaining metabolic flexibility during nutrient stress. Dysregulation of ALDH6A1 impairs amino acid and fatty acid metabolism, contributing to metabolic disorders, including methylmalonic aciduria and neurological deficits. Its activity is essential for detoxifying reactive intermediates formed during amino acid catabolism, protecting cells from oxidative stress. ALDH6A1 is particularly important in tissues with high metabolic demands, such as the liver, muscles, and brain.
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ALDH6A1 Antibody is a Rabbit Polyclonal antibody against ALDH6A1. 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.
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