Catalogue number
ENZ-907
Synonyms
MMSADHA, MMSDH , Aldehyde Dehydrogenase 6 Family, Member
A1, Methylmalonate-Semialdehyde Dehydrogenase [Acylating], Mitochondrial, Mitochondrial
Acylating Methylmalonate-Semialdehyde Dehydrogenase, Malonate-Semialdehyde
Dehydrogenase [Acylating], Aldehyde Dehydrogenase Family 6 Member A1, Malonate-Semialdehyde
Dehydrogenase, EC 1.2.1.18, EC 1.2.1.27.
Introduction
ALDH6A1
or Methylmalonate-semialdehyde
dehydrogenase [acylating], mitochondrial is a mitochondrial methylmalonate
semialdehyde dehydrogenase. ALDH6A1 participates in the valine and pyrimidine
catabolic pathways. ALDH6A1
catalyzes the irreversible oxidative decarboxylation of malonate, propionyl-CoA
and methylmalonate semialdehydes to acetyl. ALDH6A1 deficiency is distinguished
by high levels of beta-alanine, 3-hydroxypropionic acid, and the two isomers of
3-amino and 3-hydroxyisobutyric acids in urine organic acids.
Description
ALDH6A1 Human Recombinant produced in E.Coli is a single,
non-glycosylated polypeptide chain containing 525 amino acids (34-535 a.a) and
having a molecular mass of 56.8kDa. ALDH6A1 is fused to a 23 amino acid His-tag at N-terminus
& purified by proprietary chromatographic techniques.
Source
Escherichia Coli.
Physical Appearance
Sterile Filtered colorless solution.
Formulation
ALDH6A1
protein solution (0.25mg/ml) containing Phosphate buffered saline (pH7.4), 10%
glycerol and 1mM DTT.
Stability
Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time.
For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).
Avoid multiple freeze-thaw cycles.
Purity
Greater than 85.0% as determined by SDS-PAGE.
Amino acid sequence
MGSSHHHHHH
SSGLVPRGSH MGSSSSVPTV
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.
Safety Data Sheet
Usage
ProSpec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
