Rabbit anti-Human ALDH5A1 Polyclonal Antibody | anti-ALDH5A1 antibody

ALDH5A1 (Succinate-semialdehyde Dehydrogenase, Mitochondrial, Aldehyde Dehydrogenase Family 5 Member A1, NAD(+)-dependent Succinic Semialdehyde Dehydrogenase, SSADH) (PE)

Cat. Num. AAA6369516

• Gene Names: ALDH5A1; SSDH; SSADH
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ALDH5A1; Polyclonal Antibody; ALDH5A1 (Succinate-semialdehyde Dehydrogenase; Mitochondrial; Aldehyde Dehydrogenase Family 5 Member A1; NAD(+)-dependent Succinic Semialdehyde Dehydrogenase; SSADH) (PE); anti-ALDH5A1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ALDH5A1.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-ALDH5A1 antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human ALDH5A1, aa1-535 (NP_001071.1).
• Immunogen Sequence: MATCIWLRSCGARRLGSTFPGCRLRPRAGGLVPASGPAPGPAQLRCYAGRLAGLSAALLRTDSFVGGRWLPAAATFPVQDPASGAALGMVADCGVREARAAVRAAYEAFCRWREVSAKERSSLLRKWYNLMIQNKDDLARIITAESGKPLKEAHGEILYSAFFLEWFSEEARRVYGDIIHTPAKDRRALVLKQPIGVAAVITPWNFPSAMITRKVGAALAAGCTVVVKPAEDTPFSALALAELASQAGIPSGVYNVIPCSRKNAKEVGEAICTDPLVSKISFTGSTTTGKILLHHAANSVKRVSMELGGLAPFIVFDSANVDQAVAGAMASKFRNTGQTCVCSNQFLVQRGIHDAFVKAFAEAMKKNLRVGNGFEEGTTQGPLINEKAVEKVEKQVNDAVSKGATVVTGGKRHQLGKNFFEPTLLCNVTQDMLCTHEETFGPLAPVIKFDTEEEAIAIANAADVGLAGYFYSQDPAQIWRVAEQLEVGMVGVNEGLISSVECPFGGVKQSGLGREGSKYGIDEYLELKYVCYGGL
• Conjugate: PE
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of ALDH5A1 expression in transfected 293T cell line by ALDH5A1 polyclonal antibody. Lane 1: ALDH5A1 transfected lysate (57.2kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-ALDH5A1 antibody

Aldh5A1 is a member of the aldehyde dehydrogenase superfamily, a group of NAD(P)(+)-dependent enzymes that catalyze the oxidation of a wide spectrum of aliphatic and aromatic aldehydes. Aldehyde dehydrogenase enzymes are thought to play a major role in the detoxification of aldehydes generated by alcohol metabolism and lipid peroxidation. Aldh5A1 is a mitochondrial NAD(+)-dependent succinic semialdehyde dehydrogenase. A deficiency of this enzyme, known as 4-hydroxybutyricaciduria, results in a disorder of the neurotransmitter 4-aminobutyric acid (GABA). Symptoms usually include static encephalopathy, associated with developmental delays, hypotonia, ataxia, speech defects, and seizures. At least two isoforms of Aldh5A1 are known to exist.

Product Categories/Family for anti-ALDH5A1 antibody

Antibodies; Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 4507229
• NCBI GeneID: 7915
• NCBI Accession #: NP_001071.1
• NCBI GenBank Nucleotide #: NM_001080.3
• UniProt Accession #: P51649; Q546H9; Q8N3W6; B2RD26; G5E949
• Molecular Weight: 58,653 Da
• NCBI Official Full Name: succinate-semialdehyde dehydrogenase, mitochondrial isoform 2
• 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; NAD(+)-dependent succinic semialdehyde dehydrogenase; aldehyde dehydrogenase 5 family member A1; mitochondrial succinate semialdehyde dehydrogenase
• 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: Carbohydrate Metabolism - butanoate; Oxidoreductase; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 1.2.1.24; Mitochondrial

Chromosomal Location of Human Ortholog: 6p22

Cellular Component: mitochondrion; mitochondrial matrix

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

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

Disease: Succinic Semialdehyde Dehydrogenase Deficiency