ASAH1 (25-125) Mouse Monoclonal Antibody [Clone ID: 1A7]
Other products for "ASAH1"
Specifications
Product Data | |
Clone Name | 1A7 |
Applications | ELISA, IHC, WB |
Recommended Dilution | ELISA. Immunohistochemistry on Paraffin Sections: 5 µg/ml. Western Blot: 1/500 - 1/1000. |
Reactivities | Human |
Host | Mouse |
Isotype | IgG3 |
Clonality | Monoclonal |
Immunogen | ASAH1 antibody was raised against ASAH1 (NP_808592, 25 a.a. ~ 125 a.a) partial recombinant protein with GST tag |
Specificity | Recognizes Acid ceramidase ASAH1 at aa 25-125. |
Formulation | PBS, pH 7.4 State: Purified State: Liquid purified Ig fraction |
Concentration | lot specific |
Purification | Protein A Chromatography |
Storage | Upon receipt, store undiluted (in aliquots) at -20°C. Avoid repeated freezing and thawing. |
Stability | Shelf life: one year from despatch. |
Gene Name | Homo sapiens N-acylsphingosine amidohydrolase 1 (ASAH1), transcript variant 3 |
Database Link | |
Background | 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. |
Synonyms | ASAH1, ASAH, Acylsphingosine deacylase, PHP32 |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Lysosome, Metabolic pathways, Sphingolipid metabolism |
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