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

ALDH6A1, Methylmalonate-semialdehyde dehydrogenase acylating mitochondrial isoform 1, MMSADHA, MMSDH, ATGP3087-10 µg, ATGP3087-20 µg, ATGP3087-50 µg, ATGP3087-100 µg, ATGP3087-250 µg, ATGP3087-500 µg, ATGP3087-1 mg, ATGP3087-10, ATGP3087-20, ATGP3087-50, ATGP3087-100, ATGP3087-250, ATGP3087-500, ATGP3087-1

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

SSSVPTV KLFIGGKFVE SKSDKWIDIH NPATNEVIGR VPQATKAEMD AAIASCKRAF PAWADTSVLS RQQVLLRYQQ LIKENLKEIA KLITLEQGKT LADAEGDVFR GLQVVEHACS VTSLMMGETM PSITKDMDLY SYRLPLGVCA GIAPFNFPAM IPLWMFPMAM VCGNTFLMKP SERVPGATML LAKLLQDSGA PDGTLNIIHG QHEAVNFICD HPDIKAISFV GSNKAGEYIF ERGSRHGKRV QANMGAKNHG VVMPDANKEN TLNQLVGAAF GAAGQRCMAL STAVLVGEAK KWLPELVEHA KNLRVNAGDQ PGADLGPLIT PQAKERVCNL IDSGTKEGAS ILLDGRKIKV KGYENGNFVG PTIISNVKPN MTCYKEEIFG PVLVVLETET LDEAIQIVNN NPYGNGTAIF TTNGATARKY AHLVDVGQVG VNVPIPVPLP MFSFTGSRSS FRGDTNFYGK QGIQFYTQLK TITSQWKEED ATLSSPAVVM PTMGR