ALDH5A1 blocking peptide

ALDH5A1 Antibody (N-term) Blocking Peptide

• Gene Names: ALDH5A1; SSDH; SSADH
• Synonyms: ALDH5A1; ALDH5A1 Antibody (N-term) Blocking Peptide; Succinate-semialdehyde dehydrogenase; mitochondrial; Aldehyde dehydrogenase family 5 member A1; NAD(+)-dependent succinic semialdehyde dehydrogenase; SSADH; ALDH5A1 blocking peptide

• Specificity: The synthetic peptide sequence used to generate the antibody was selected from the N-term region of human ALDH5A1. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 535

• Cellular Location: Mitochondrion.
• Tissue Location: Brain, pancreas, heart, liver, skeletal muscle and kidney. Lower in placenta
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for ALDH5A1 blocking peptide

Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).

NCBI and Uniprot Product Information

• NCBI GI #: 7531278
• NCBI GeneID: 7915
• UniProt Accession #: P51649; Q546H9; Q8N3W6; B2RD26; G5E949
• Molecular Weight: 58,653 Da
• NCBI Official Full Name: Succinate-semialdehyde dehydrogenase, mitochondrial
• NCBI Official 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 Gene Name: ALDH5A1
• UniProt Synonym Gene Names: SSADH
• UniProt Entry Name: SSDH_HUMAN

NCBI Description

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 Description

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.

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