84-1336-07　ASAH1 (Acid Ceramidase, AC, ACDase, Acid CDase, Acylsphingosine Deacylase, N-acylsphingosine Amidohydrolase, Putative 32kD Heart Protein, PHP32, ASAH, HSD33, HSD-33) 100ug　123590

特徴

• 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.
• Applications:Suitable for use in Western Blot.
• Other applications not tested.
• Recommended Dilutions:Optimal dilutions to be determined by the researcher.
• Amno Acid Sequence:MPGRSCVALVLLAAAVSCAVAQHAPPWTEDCRKSTYPPSGPTYRGAVPWYTINLDLPPYKRWHELMLDKAPMLKVIVNSLKNMINTFVPSGKVMQVVDEKLPGLLGNFPGPFEEEMKGIAAVTDIPLGEIISFNIFYELFTICTSIVAEDKKGHLIHGRNMDFGVFLGWNINNDTWVITEQLKPLTVNLDFQRNNKTVFKASSFAGYVGMLTGFKPGLFSLTLNERFSINGGYLGILEWILGKKDAMWIGFLTRTVLENSTSYEEAKNLLTKTKILAPAYFILGGNQSGEGCVITRDRKESLDVYELDAKQGRWYVVQTNYDRWKHPFFLDDRRTPAKMCLNRTSQENISFETMYDVLSTKPVLNKLTVYTTLIDVTKGQFETYLRDCPDPCIGW
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.