Catalog# : 4741
Sphingolipids are hydrolyzed by ceramidases to yield sphingosine and fatty acids. These ceramidases are classified according to the pH range that supports their optimal activity. ASAH1 is an acid ceramidase and key regulator of ceramide metabolism. Mutations in this gene results in Farber Lipogranulomatosis, a fatal human genetic disorder that results in the painful swelling of the joints and tendons and pulminary insufficiency, while a complete knockout of its expression is lethal in mice. Recent studies have shown elevated levels of ASAH1 in Alzheimer’s disease (AD) patients correlating with a reduction in sphingomyelin and elevation of ceramide. Pretreatment of cultured neurons with recombinant AHAH1 prevented the cells from undergoing A-beta (Ab)-induced apoptosis. Multiple isoforms of this protein are known to exist.
Additional Names : ASAH1, N-acylsphingosine amidohydrolase (non-lysosomal ceramidase) 1, acid ceramidase
DescriptionLeft: Western blot analysis of ASAH1 in mouse heart tissue lysate with ASAH1 antibody at (A) 1 and (B) 2 µg/ml.
Below: Immunohistochemistry of ASAH1 in human heart tissue with ASAH1 antibody at 2.5 μg/ml.
Other Product Images
Source : ASAH1 antibody was raised against a 16 amino acid peptide near the carboxy terminus of the human ASAH1.Purification : Affinity chromatography purified via peptide column
Clonality and Clone : This is a polyclonal antibody.
Host : ASAH1 antibody was raised in rabbit. Please use anti-rabbit secondary antibodies.
Application : ASAH1 antibody can be used for detection of ASAH1 by Western blot at 1 µg/ml.
Tested Application(s) : E, WB
Buffer : Antibody is supplied in PBS containing 0.02% sodium azide.
Blocking Peptide : Cat.No. 4741P - ASAH1 Peptide
Long-Term Storage : ASAH1 antibody can be stored at 4ºC, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Positive Control
1. Cat. No. 1401 - Mouse Heart Tissue Lysate
Species Reactivity :H, M, R
GI Number : 119584199
Accession Number : EAW63795
Short Description : an acid ceramidase
References
1. Nilsson A and Duan RD. Alkaline sphingomyelinases and ceramidases of the gastrointestinal tract. Chem. Phys. Lipids 1999; 102:97-105.
2. Koch J, Gartner S, Li CM, et al. Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification of the first molecular lesion causing Farber’s disease. J. Biol. Chem. 1996; 271:33110-5.
3. Li CM, Park JH, Simonaro CM, et al. Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes. Genomics 2002; 79:218-24.
4. He X, Huang Y, Li B. Deregulation of sphingolipid metabolism in Alzheimer’s disease. Neurobiol. Aging 2008; epub.
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