N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) (AA 88-182) antibody

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

Synonyms: MGC82286, asah1, MGC173782, zgc:101637, ASAH1, MGC140781, DKFZp469G2224, AC, PHP, ASAH, PHP32, FLJ21558, FLJ22079, Asah, AL022942, AU044555, 2310081N20Rik

Binding Site: AA 88-182

Clonality: Monoclonal (23)

Host: Mouse

Reactivity: Human

Application: Western Blotting (WB)

Catalog no.: ABIN968809

Additional Information

Alternative name: Acid Ceramidase

Immunogen: Human Acid Ceramidase

Cross-Reactivity: Dog (Canine)

Format: Liquid

Isotype: IgG1

Clone: 23

Description: Ceramide is a sphingolipid that exhibits a wide variety of functions, including monocyte differentiation, apoptosis, neurite outgrowth, and Ca2+ transport. It also serves as the precursor of many sphingolipids and anchors these into the outer leaflet of the plasma membrane via hydrophobic interactions. Acid ceramidase is a lysosomal enzyme that was purified from human urine. It is synthesized as a 55kDa precursor protein, which is then processesed into the mature alpha-subunit (13kDa) and beta-subunit (40kDa). Acid ceramidase catalyzes the hydrolysis of ceramide into free fatty acid and sphingosine. Sphingosine, and its phosphorylated form, sphingosine-1-phosphate (SPP), have been shown to inhibit PKC activity and act as a second messenger in cell proliferation and differentiation. Acid ceramidase is also the cause of a lysosomal storage disorder known as Farber’s disease. This disease is characterized by an accumulation of ceramide in tissues, leading to swelling and pain of the joints and extremities, pulmonary insufficiency, and death at an early age. Thus, acid ceramidase is necessary in the hydrolysis of ceramide and is the cause of Farber’s disease.

Characteristics: 1. Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2. Please refer to us for technical protocols.
3. Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
4. Source of all serum proteins is from USDA inspected abattoirs located in the United States.

Molecular Weight: 13 kDa

Application Details

Concentration: 250 µg/ml

Purity: Purified

Purification: Purified from tissue culture supernatant or ascites by affinity chromatography.

Buffer: Aqueous buffered solution containing BSA, glycerol.

Preservative: 0.09% Sodium azide.

Storage: Store undiluted at -20°C.

Restrictions: For Research Use only

Images

Western blot analysis of Acid Ceramidase on NHEK lysate. Lane 1: 1:250, lane 2: 1:500, lane 3: 1:1000 dilution of Acid Ceramidase.

Publications

Product: Bernardo, Hurwitz, Zenk et al.: "Purification, characterization, and biosynthesis of human acid ceramidase." in: The Journal of biological chemistry, Vol. 270, Issue 19, pp. 11098-102, 1995 (PubMed).

Strelow, Bernardo, Adam-Klages et al.: "Overexpression of acid ceramidase protects from tumor necrosis factor-induced cell death." in: The Journal of experimental medicine, Vol. 192, Issue 5, pp. 601-12, 2000 (PubMed).

Ferlinz, Kopal, Bernardo et al.: "Human acid ceramidase: processing, glycosylation, and lysosomal targeting." in: The Journal of biological chemistry, Vol. 276, Issue 38, pp. 35352-60, 2001 (PubMed).