Cell Biology Antibodies 6
Anti-ALDH5A1 Antibody (CAB16074)
- SKU:
- CAB16074
- Product Type:
- Antibody
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
| Antibody Name: | Anti-ALDH5A1 Antibody |
| Antibody SKU: | CAB16074 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 400-500 of human ALDH5A1 (NP_001071.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Mouse, Rat |
| Positive Samples: | Rat brain |
| Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 400-500 of human ALDH5A1 (NP_001071.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | VSKG ATVV TGGK RHQL GKNF FEPT LLCN VTQD MLCT HEET FGPL APVI KFDT EEEA IAIA NAAD VGLA GYFY SQDP AQIW RVAE QLEV GMVG VNEG LISS V |
| Gene ID: | 7915 |
| Uniprot: | P51649 |
| Cellular Location: | Mitochondrion |
| Calculated MW: | 57kDa/58kDa |
| Observed MW: | 57kDa |
| Synonyms: | ALDH5A1, SSADH, SSDH |
| Background: | 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. |
| 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 |
