Human ALDH5A1 Recombinant Protein (RPPB1389)

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SKU:
RPPB1389
Product type:
Recombinant Protein
Size:
10ug
Species:
Human
Target:
ALDH5A1
Synonyms:
Succinate-semialdehyde dehydrogenase mitochondrial
Synonyms:
Aldehyde dehydrogenase family 5 member A1
Synonyms:
NAD(+)-dependent succinic semialdehyde dehydrogenase
Synonyms:
ALDH5A1
Source:
Escherichia Coli
Uniprot:
P51649
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Description

Product Name:Human ALDH5A1 Recombinant Protein
Product Code:RPPB1389
Size:10µg
Species:Human
Target:ALDH5A1
Synonyms:Succinate-semialdehyde dehydrogenase mitochondrial, Aldehyde dehydrogenase family 5 member A1, NAD(+)-dependent succinic semialdehyde dehydrogenase, ALDH5A1, SSADH, SSDH.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The ALDH5A1 solution (0.5 mg/ml) contains 20mM Tris-HCl buffer(pH 8.0), 10% glycerol 1mM DTT, 0.1M NaCl and 1mM EDTA.
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 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MAGRLAGLSA ALLRTDSFVG GRWLPAAATF PVQDPASGAA LGMVADCGVR EARAAVRAAY EAFCRWREVS AKERSSLLRK WYNLMIQNKD DLARIITAES GKPLKEAHGE ILYSAFFLEW FSEEARRVYG DIIHTPAKDR RALVLKQPIG VAAVITPWNF PSAMITRKVG AALAAGCTVV VKPAEDTPFS ALALAELASQ AGIPSGVYNV IPCSRKNAKE VGEAICTDPL VSKISFTGST TTGKILLHHA ANSVKRVSME LGGLAPFIVF DSANVDQAVA GAMASKFRNT GQTCVCSNQF LVQRGIHDAF VKAFAEAMKK NLRVGNGFEE GTTQGPLINE KAVEKVEKQV NDAVSKGATV VTGGKRHQLG KNFFEPTLLC NVTQDMLCTH EETFGPLAPV IKFDTEEEAI AIANAADVGL AGYFYSQDPA QIWRVAEQLE VGMVGVNEGL ISSVECPFGG VKQSGLGREG SKYGIDEYLE LKYVCYGGL

ALDH5A1 is a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase, which is a member of the aldehyde dehydrogenase family of proteins. The ALDH5A1 protein functions as a mediator to the NADP+-dependent oxidation of aldehydes into acids and has an imperative role in the detoxification of alcohol-derived acetaldehyde, as well as in lipid peroxidation and in the metabolism of corticosteroids, biogenic amines and neurotransmitters. ALDH5A1 is expressed in various tissues, including the liver, heart, lung, brain, kidney and placenta. Deficiency in the ALDH5A1 enzyme, known as 4-hydroxybutyricaciduria, is a rare inborn error in the metabolism of the neurotransmitter 4-aminobutyric acid (GABA). In response to this defect, physiologic fluids from patients accumulate GHB, which is a compound with numerous neuromodulatory properties.

ALDH5A1 Human Recombinant fused with a 20 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 509 amino acids (48-535 a.a.) and having a molecular mass of 54.6kDa. The ALDH5A1 is purified by proprietary chromatographic techniques.

UniProt Protein Function:ALDH5A1: Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA). Defects in ALDH5A1 are the cause of succinate semialdehyde dehydrogenase deficiency (SSADH deficiency). SSADH deficiency is a rare inborn error in the metabolism of 4-aminobutyric acid (GABA) which leads to accumulation of 4-hydroxybutyric acid in physiologic fluids of patients. The disease is characterized by severe ataxia and by mildly retarded psychomotor development. Belongs to the aldehyde dehydrogenase family.
UniProt Protein Details:

Protein type:EC 1.2.1.24; Oxidoreductase; Mitochondrial; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - alanine, aspartate and glutamate

Chromosomal Location of Human Ortholog: 6p22

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function:aldehyde dehydrogenase (NAD) activity; protein homodimerization activity; succinate-semialdehyde dehydrogenase [NAD(P)+] activity; succinate-semialdehyde dehydrogenase activity

Biological Process: acetate metabolic process; central nervous system development; galactosylceramide metabolic process; gamma-aminobutyric acid catabolic process; glucose metabolic process; glutamate metabolic process; glutamine metabolic process; glutathione metabolic process; glycerophospholipid metabolic process; neurotransmitter catabolic process; protein homotetramerization; short-chain fatty acid metabolic process; succinate metabolic process

Disease: Succinic Semialdehyde Dehydrogenase Deficiency

NCBI Summary:This protein belongs to the aldehyde dehydrogenase family of proteins. This gene encodes a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase. A deficiency of this enzyme, known as 4-hydroxybutyricaciduria, is a rare inborn error in the metabolism of the neurotransmitter 4-aminobutyric acid (GABA). In response to the defect, physiologic fluids from patients accumulate GHB, a compound with numerous neuromodulatory properties. Two transcript variants encoding distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:P51649
NCBI GenInfo Identifier:7531278
NCBI Gene ID:7915
NCBI Accession:P51649.2
UniProt Secondary Accession:P51649,Q546H9, Q8N3W6, B2RD26, G5E949,
UniProt Related Accession:P51649
Molecular Weight:58,653 Da
NCBI Full Name:Succinate-semialdehyde dehydrogenase, mitochondrial
NCBI Synonym Full Names:aldehyde dehydrogenase 5 family member A1
NCBI Official Symbol:ALDH5A1  
NCBI Official Synonym Symbols:SSDH; SSADH  
NCBI Protein Information:succinate-semialdehyde dehydrogenase, mitochondrial
UniProt Protein Name:Succinate-semialdehyde dehydrogenase, mitochondrial
UniProt Synonym Protein Names:Aldehyde dehydrogenase family 5 member A1; NAD(+)-dependent succinic semialdehyde dehydrogenase
UniProt Gene Name:ALDH5A1  
UniProt Entry Name:SSDH_HUMAN
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