The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml. Best used within three months from the date of receipt.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.3% Glutathione, 0.79% Tris HCl
Acid ceramidase precursor
Acid ceramidase subunit beta
N acylsphingosine amidohydrolase
N acylsphingosine amidohydrolase (acid ceramidase) 1
N acylsphingosine amidohydrolase 1
Putative 32 kDa heart protein
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid.
Broadly expressed with highest expression in heart.
Involvement in disease
Defects in ASAH1 are the cause of Farber lipogranulomatosis (FL) [MIM:228000]; also known as Farber disease (FD). This sphingolipid disease is characterized by subcutaneous lipid-loaded nodules, excruciating pain in the joints and extremities, marked accumulation of ceramide in lysosomes, and death by three years of age.