P5CS anticorps (AA 1-224)

N° du produit ABIN7599110

L’anticorps Lapin Polyclonal anti-P5CS a été validé pour WB et ELISA. Il convient pour détecter P5CS dans des échantillons de Humain, Souris et Rat.

Aperçu rapide pour P5CS anticorps (AA 1-224) (ABIN7599110)

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

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp P5CS est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-P5CS anticorps

Épitope: AA 1-224

Fonction: Anti-P5CS/ALDH18A1 Antibody Picoband®

Réactivité croisée (Details): No cross-reactivity with other proteins.

Attributs du produit: 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.

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

Isotype: IgG

Information d'application

Indications d'application: 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

Stockage

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.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Détails sur P5CS

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

Autre désignation: ALDH18A1

Sujet:

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.

Poids moléculaire: 87 kDa

ID gène: 5832

UniProt: P54886