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Succinate-semialdehyde dehydrogenase, Polyclonal Antibody

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产品名称: Succinate-semialdehyde dehydrogenase, Polyclonal Antibody
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简单介绍

Succinate-semialdehyde dehydrogenase, Polyclonal Antibody


Succinate-semialdehyde dehydrogenase, Polyclonal Antibody  的详细介绍
Product Name

Succinate-semialdehyde dehydrogenase (ALDH5A1), Polyclonal Antibody

Full Product Name

Rabbit anti-human Succinate-semialdehyde dehydrogenase, mitochondrial polyclonal Antibody, Biotin conjugated

Product Synonym Names
Aldehyde dehydrogenase family 5 member A1; NAD(+)-dependent succinic semialdehyde dehydrogenase; SSADH; ALDH5A1
Product Gene Name

anti-ALDH5A1 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
271980
3D Structure
ModBase 3D Structure for P51649
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
Caprylic Acid Ammonium Sulfate Precipitation Purified
Storage Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Conjugate
Biotin
Immunogen
Recombinant human Succinate-semialdehyde dehydrogenase, mitochondrial protein(48-535AA)
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-70004 / sc-70007 / sc-130684
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-ALDH5A1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-ALDH5A1 antibody
Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Applications Tested/Suitable for anti-ALDH5A1 antibody
ELISA (EIA)
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NCBI/Uniprot data below describe general gene information for ALDH5A1. It may not necessarily be applicable to this product.
NCBI GI #
4507229
NCBI GeneID
7915
NCBI Accession #
NP_001071.1 [Other Products]
NCBI GenBank Nucleotide #
NM_001080.3 [Other Products]
UniProt Primary Accession #
P51649 [Other Products]
UniProt Secondary Accession #
Q546H9; Q8N3W6; B2RD26; G5E949[Other Products]
UniProt Related Accession #
P51649[Other Products]
Molecular Weight
58,653 Da[Similar Products]
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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  [Similar Products]
NCBI Official Synonym Symbols
SSDH; SSADH
  [Similar Products]
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
Protein Family
Succinate-semialdehyde dehydrogenase
UniProt Gene Name
ALDH5A1  [Similar Products]
UniProt Synonym Gene Names
SSADH  [Similar Products]
UniProt Entry Name
SSDH_HUMAN
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NCBI Summary for ALDH5A1
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]
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UniProt Comments for ALDH5A1
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; EC 1.2.1.24; Mitochondrial; Amino Acid Metabolism - alanine, aspartate and glutamate

Chromosomal Location of Human Ortholog: 6p22

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: aldehyde dehydrogenase (NAD) activity; carboxylic acid binding; NAD binding; 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; glucosylceramide metabolic process; glutamate metabolic process; glutamine metabolic process; glutathione metabolic process; glycerophospholipid metabolic process; neurotransmitter catabolic process; neurotransmitter secretion; post-embryonic development; protein homotetramerization; short-chain fatty acid metabolic process; succinate metabolic process; synaptic transmission

Disease: Succinic Semialdehyde Dehydrogenase Deficiency
Research Articles on ALDH5A1
1. T Change in protein: p.L138F EXON: 4 Nucleotide change: c.754G>T Change in protein: p.Q252X EXON: 8 Nucleotide change: c.1360G>A Change in protein: p.A454T">Pearl et al. identify 3 new pathogenic mutations in the ALDH5A1 gene previously unreported in the literature. EXON: 1 Nucleotide change: c.412 C>T Change in protein: p.L138F EXON: 4 Nucleotide change: c.754G>T Change in protein: p.Q252X EXON: 8 Nucleotide change: c.1360G>A Change in protein: p.A454T
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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