ASAH1 anticorps (AA 63-393)

N° du produit ABIN7602206

Cet anticorps anti-ASAH1 est un anticorps Lapin Polyclonal détectant ASAH1 dans WB, IHC, ELISA, ICC et IF. Adapté pour Humain, Souris et Rat.

Aperçu rapide pour ASAH1 anticorps (AA 63-393) (ABIN7602206)

Antigène: ASAH1 (N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp ASAH1 est non-conjugé

Application: Western Blotting (WB), Immunohistochemistry (IHC), ELISA, Immunocytochemistry (ICC), Immunofluorescence (IF)

Détail du produit anti-ASAH1 anticorps

Épitope: AA 63-393

Fonction: Anti-ASAH1 Picoband® Antibody

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

Attributs du produit: Anti-ASAH1 Picoband® Antibody (ABIN7602206). Tested in ELISA, IF, IHC, ICC, 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 ASAH1 recombinant protein (Position: H63-I393).

Isotype: IgG

Information d'application

Indications d'application: Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human, Mouse
Immunocytochemistry/Immunofluorescence, 2 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
1. Alves, M. Q., Le Trionnaire, E., Ribeiro, I., Carpentier, S., Harzer, K., Levade, T., Ribeiro, M. G. Molecular basis of acid ceramidase deficiency in a neonatal form of Farber disease: identification of the first large deletion in ASAH1 gene. Molec. Genet. Metab. 109: 276-281, 2013. 2. Souillet, G., Guibaud, P., Fensom, A. H., Maire, I., Zabot, M. T. Outcome of displacement bone marrow transplantation in Farber's disease: a report of a case.In: Hobbs, J. R. (ed.) : Correction of Certain Genetic Diseases by Transplantation. London: COGENT 1989. Pp. 137-141. 3. Zhou, J., Tawk, M., Tiziano, F. D., Veillet, J., Bayes, M., Nolent, F., Garcia, V., Servidei, S., Bertini, E., Castro-Giner, F., Renda, Y., Carpentier, S., Andrieu-Abadie, N., Gut, I., Levade, T., Topaloglu, H., Melki, J. Spinal muscular atrophy associated with progressive myoclonic epilepsy is caused by mutations in ASAH1. Am. J. Hum. Genet. 91: 5-14, 2012.

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, 0.05 mg NaN₃.

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 ASAH1

Antigène: ASAH1 (N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1))

Autre désignation: ASAH1

Sujet:

Synonyms: Acid ceramidase, AC, ACDase, Acid Cdase, Acylsphingosine deacylase, N-acylethanolamine hydrolase ASAH1, N-acylsphingosine amidohydrolase, Putative 32 kDa heart protein, PHP32, Acid ceramidase subunit alpha, Acid ceramidase subunit beta, ASAH1, ASAH, HSD-33, HSD33

Tissue Specificity: Widely distributed throughout the CNS. Particularly abundant in kidney and intestinal microvilli, also detected in lung and liver. Weakly expressed in heart and aorta.

Background: The ASAH1 gene encodes in humans the acid ceramidase enzyme. It is mapped to 8p22. This gene encodes a member of the acid ceramidase family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. Processing of this preproprotein generates alpha and beta subunits that heterodimerize to form the mature lysosomal enzyme, which catalyzes the degradation of ceramide into sphingosine and free fatty acid. This enzyme is overexpressed in multiple human cancers and may play a role in cancer progression. Mutations in this gene are associated with the lysosomal storage disorder, Farber lipogranulomatosis, and a neuromuscular disorder, spinal muscular atrophy with progressive myoclonic epilepsy.

Poids moléculaire: 45 kDa

ID gène: 427

UniProt: Q13510