Recombinant human ALDH6A1 protein
ALDH6A1 also known as Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial. ALDH6A1 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. ALDH6A1 deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids. Recombinant human ALDH6A1, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.
Product Specifications
Product Name Alternative
Methylmalonate-semialdehyde dehydrogenase acylating mitochondrial isoform 1, MMSADHA, MMSDH
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE
Concentration
0.25 mg/mL (determined by Bradford assay)
Purity
> 85% by SDS-PAGE
Molecular Weight
56.8 kDa (525aa)
Additionnal Information
References & Citations
Sass JO., et al. (2012) J. Inherit. Metab. Dis. 35 (3), 437-442
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. Phosphate-Buffered Saline (pH 7.4) containing 10% glycerol, 1mM DTT
Scientific Category
Signal Transduction
NCBI Accession Number
NP_005580
Uniprot Accession Number
Q02252
Species
Human
AA Sequence
Frequently Asked Questions
Explore Other Products
Browse additional items from our catalog
