Rabbit anti-Human, Mouse ALDH18A1 Polyclonal Antibody | anti-ALDH18A1 antibody

ALDH18A1 Polyclonal Antibody

Cat. Num. AAA3007702

• Gene Names: ALDH18A1; GSAS; P5CS; PYCS; SPG9; ADCL3; SPG9A; SPG9B; ARCL3A
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ALDH18A1; Polyclonal Antibody; ALDH18A1 Polyclonal Antibody; Delta-1-pyrroline-5-carboxylate synthase; P5CS; Aldehyde dehydrogenase family 18 member A1; Glutamate 5-kinase; GK; Gamma-glutamyl kinase; Gamma-glutamyl phosphate reductase; GPR; Glutamate-5-semialdehyde dehydrogenase; Glutamyl-gamma-semialdehyde dehydrogenase; GSAS; PYCS; anti-ALDH18A1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ALDH18A1 pAb detects endogenous levels of ALDH18A1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 795

• Applicable Applications for anti-ALDH18A1 antibody: Western Blot (WB)
• Application Notes: WB: 1:2000-1:10,000
• Immunogen: Synthetic peptide, corresponding to Human ALDH18A1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of ALDH18A1 pAb at 1:2000 dilutionLane1:The Embryo tissue lysate of Mouse (40ug) Lane2:A375 whole cell lysate (40ug) Lane3:HCT116 whole cell lysate (20ug))

Related Product Information for anti-ALDH18A1 antibody

Aldehyde dehydrogenases (ALDHs) mediate NADP+-dependent oxidation of aldehydes into acids during the detoxification of alcohol-derived acetaldehyde, lipid peroxidation, and metabolism of corticosteroids, biogenic amines and neurotransmitters. ALDH18A1 (aldehyde dehydrogenase 18 family member A1), also known as GSAS (glutamate gamma-semialdehyde synthetase), P5CS (delta1- pyrroline-5-carboxylate synthetase) or PYCS, is the major enzyme involved in proline, arginine and ornithine biosynthesis. Localizing to the inner mitochondrial membrane, ALDH18A1 catalyzes the reduction of glutamate to delta1- pyrroline-5-carboxylate. Due to alternative splicing events, two isoforms exist for ALDH18A1, differing by only two amino acids. The longer isoform is widely expressed while the shorter isoform predominantly functions in the gut. A mutation in the gene encoding ALDH18A1 results in P5CS deficiency, a disease characterized by progressive joint laxity, neurodegeneration, bilateral subcapsular cataracts and skin hyperelasticity.

NCBI and Uniprot Product Information

• NCBI GI #: 6226882
• NCBI GeneID: 5832
• UniProt Accession #: P54886
• Molecular Weight: ~ 87kDa
• NCBI Official Full Name: Delta-1-pyrroline-5-carboxylate synthase
• NCBI Official Synonym Full Names: aldehyde dehydrogenase 18 family member A1
• NCBI Official Symbol: ALDH18A1
• NCBI Official Synonym Symbols: GSAS; P5CS; PYCS; SPG9; ADCL3; SPG9A; SPG9B; ARCL3A
• NCBI Protein Information: delta-1-pyrroline-5-carboxylate synthase
• UniProt Protein Name: Delta-1-pyrroline-5-carboxylate synthase
• UniProt Gene Name: ALDH18A1
• UniProt Synonym Gene Names: GSAS; P5CS; PYCS; P5CS; GK; GPR
• UniProt Entry Name: P5CS_HUMAN

NCBI Description

This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

ALDH18A1: Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine. Defects in ALDH18A1 are the cause of cutis laxa, autosomal recessive, type 3A (ARCL3A). A syndrome characterized by facial dysmorphism with a progeroid appearance, large and late-closing fontanel, cutis laxa, joint hyperlaxity, athetoid movements and hyperreflexia, pre- and postnatal growth retardation, intellectual deficit, developmental delay, and ophthalmologic abnormalities. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Oxidoreductase; EC 2.7.2.11; Amino Acid Metabolism - arginine and proline; EC 1.2.1.41; Mitochondrial; Kinase, other

Chromosomal Location of Human Ortholog: 10q24.3

Cellular Component: mitochondrion; cytoplasm; mitochondrial inner membrane

Molecular Function: glutamate 5-kinase activity; glutamate-5-semialdehyde dehydrogenase activity; ATP binding

Biological Process: citrulline biosynthetic process; glutamate metabolic process; proline biosynthetic process; ornithine biosynthetic process; amino acid biosynthetic process; phosphorylation

Disease: Cutis Laxa, Autosomal Recessive, Type Iiia