Anti-ALDH6A1 Antibody Picoband®

SKU: A08868-1

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC

Application Details: Western blot, 0.1-0.25μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml, Human

Gene Name: ALDH6A1

Specificity:

Background: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial (MMSDH) is an enzyme that in humans is encoded by the ALDH6A1 gene. 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: A synthetic peptide corresponding to a sequence at the C-terminus of human ALDH6A1, identical to the related mouse and rat sequences.

Clonality: Polyclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Chambliss, K. L., Gray, R. G. F., Rylance, G., Pollitt, R. J., Gibson, K. M. Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency. J. Inherit. Metab. Dis. 23: 497-504, 2000. 2. Dobrowolski, S. F., Alodaib, A., Karunanidhi, A., Basu, S., Holecko, M., Lichter-Konecki, U., Pappan, K. L., Vockley, J. Clinical, biochemical, mitochondrial, and metabolomic aspects of methylmalonate semialdehyde dehydrogenase deficiency: report of a fifth case. Molec. Genet. Metab. 129: 272-277, 2020. 3. Gray, R. G. F., Pollitt, R. J., Webley, J. Methylmalonic semialdehyde dehydrogenase deficiency: demonstration of defective valine and beta-alanine metabolism and reduced malonic semialdehyde dehydrogenase activity in cultured fibroblasts. Biochem. Med. Metab. Biol. 38: 121-124, 1987.

Uniprot ID: Q02252

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 58 kDa

Calculated Molecular Weight:

Gene ID: 4329

Protein Name: Methylmalonate-semialdehyde/malonate-semialdehyde dehydrogenase [acylating], mitochondrial

Gene Full Name: aldehyde dehydrogenase 6 family member A1

Synonyms: ALDH6A1; MMSADHA; MMSDH