P5CS antibody (AA 1-224)

Catalog No. ABIN7599110

The Rabbit Polyclonal anti-P5CS antibody has been validated for WB and ELISA. It is suitable to detect P5CS in samples from Human, Mouse and Rat.

Quick Overview for P5CS antibody (AA 1-224) (ABIN7599110)

Target: P5CS (ALDH18A1) (Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This P5CS antibody is un-conjugated

Application: Western Blotting (WB), ELISA

Product Details anti-P5CS Antibody

Binding Specificity: AA 1-224

Purpose: Anti-P5CS/ALDH18A1 Antibody Picoband®

Cross-Reactivity (Details): No cross-reactivity with other proteins.

Characteristics: Anti-P5CS/ALDH18A1 Antibody Picoband® (ABIN7599110). Tested in ELISA, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Purification: Immunogen affinity purified.

Immunogen: E.coli-derived human P5CS/ALDH18A1 recombinant protein (Position: M1-A224).

Isotype: IgG

Application Details

Application Notes: Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
ELISA, 0.1-0.5 μg/mL, -
1. Bicknell, L. S., Pitt, J., Aftimos, S., Ramadas, R., Maw, M. A., Robertson, S. P. A missense mutation in ALDH18A1, encoding delta-1-pyrroline-5-carboxylate synthase (P5CS), causes an autosomal recessive neurocutaneous syndrome. Europ. J. Hum. Genet. 16: 1176-1186, 2008. 2. Fischer, B., Callewaert, B., Schroter, P., Coucke, P. J., Schlack, C., Ott, C.-E., Morroni, M., Homann, W., Mundlos, S., Morava, E., Ficcadenti, A., Kornak, U. Severe congenital cutis laxa with cardiovascular manifestations due to homozygous deletions in ALDH18A1. Molec. Genet. Metab. 112: 310-316, 2014. 3. Panza, E., Escamilla-Honrubia, J. M., Marco-Marin, C., Gougeard, N., De Michele, G., Brescia Morra, V., Liguori, R., Salviati, L., Donati, M. A., Cusano, R., Pippucci, T., Ravazzolo, R., Nemeth, A. H., Smithson, S., Davies, S., Hurst, J. A., Bordo, D., Rubio, V., Seri, M. ALDH18A1 gene mutations cause dominant spastic paraplegia SPG9: loss of function effect and plausibility of a dominant negative mechanism. (Letter) Brain 139: e3, 2016. Note: Electronic Article.

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg Sodium azide.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Target Details for P5CS

Target: P5CS (ALDH18A1) (Aldehyde Dehydrogenase 18 Family, Member A1 (ALDH18A1))

Alternative Name: ALDH18A1

Background:

Synonyms: Delta-1-pyrroline-5-carboxylate synthase, P5CS, Aldehyde dehydrogenase family 18 member A1, GK, Gamma-glutamyl kinase, GPR, Glutamate-5-semialdehyde dehydrogenase, Glutamyl-gamma-semialdehyde dehydrogenase, ALDH18A1, GSAS, P5CS, PYCS

Tissue Specificity: Detected in pancreas islets (at protein level). Detected in brain and pancreas.

Background: Delta-1-pyrroline-5-carboxylate synthetase (P5CS) is an enzyme that in humans is encoded by the ALDH18A1 gene. This gene is mapped to 10q24.1. 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.

Molecular Weight: 87 kDa

Gene ID: 5832

UniProt: P54886