Human SSADH ELISA Kit | SSADH elisa kit

Human SSADH (Succinate-semialdehyde dehydrogenase, mitochondrial) ELISA Kit

Cat. Num. AAA2516109

• Gene Names: ALDH5A1; SSDH; SSADH
• Reactivity: Human
• Synonyms: SSADH; Human SSADH (Succinate-semialdehyde dehydrogenase; mitochondrial) ELISA Kit; SSADH elisa kit

• Reactivity: Human
• Specificity: This kit recognizes Human SSADH in samples. No significant cross-reactivity or interference between Human SSADH and analogues was observed.
• Sequence Length: 535

• Samples: Serum, plasma and other biological fluids
• Assay Type: Quantitative Sandwich
• Detection Range: 31.25-2000pg/mL
• Sensitivity: 18.75pg/mL
• Intra-assay Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, mid range and high level Human SSADH were tested 20 times on one plate, respectively.
• Inter-assay Precision: Inter-assay Precision (Precision between assays): 3 samples with low, mid range and high level Human SSADH were tested on 3 different plates, 20 replicates in each plate.
• Preparation and Storage: Store at 4 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for SSADH elisa kit

Intended Uses: This ELISA kit applies to the in vitro quantitative determination of Human SSADH concentrations in serum, plasma and other biological fluids.

Principle of the Assay: This ELISA kit uses the Sandwich-ELISA principle. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to Human SSADH. Standards or samples are added to the micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for Human SSADH and Avidin-Horseradish Peroxidase (HRP) conjugate are added successively to each micro plate well and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain Human SSADH, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of stop solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of Human SSADH. You can calculate the concentration of Human SSADH in the samples by comparing the OD of the samples to the standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 4507229
• NCBI GeneID: 7915
• NCBI Accession #: NP_001071.1
• NCBI GenBank Nucleotide #: NM_001080.3
• 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: Oxidoreductase; Mitochondrial; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - alanine, aspartate and glutamate; EC 1.2.1.24

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; glutamine metabolic process; glucosylceramide metabolic process; post-embryonic development; protein homotetramerization; synaptic transmission; glutathione metabolic process; galactosylceramide metabolic process; glycerophospholipid metabolic process; gamma-aminobutyric acid catabolic process

Disease: Succinic Semialdehyde Dehydrogenase Deficiency