ASAH1 Antikörper (AA 63-393)

Produktnummer ABIN7602206

Dieses Anti-ASAH1-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von ASAH1 in WB, IHC, ELISA, ICC und IF. Geeignet für Human, Maus und Ratte.

Kurzübersicht für ASAH1 Antikörper (AA 63-393) (ABIN7602206)

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

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser ASAH1 Antikörper ist unkonjugiert

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

Produktdetails anti-ASAH1 Antikörper

Bindungsspezifität: AA 63-393

Verwendungszweck: Anti-ASAH1 Picoband® Antibody

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

Produktmerkmale: 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.

Aufreinigung: Immunogen affinity purified.

Immunogen: E.coli-derived human ASAH1 recombinant protein (Position: H63-I393).

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: 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.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

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

Konzentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na₂HPO₄, 0.05 mg NaN₃.

Konservierungsmittel: Sodium azide

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Details zu ASAH1

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

Andere Bezeichnung: ASAH1

Hintergrund:

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.

Molekulargewicht: 45 kDa

Gen-ID: 427

UniProt: Q13510