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

Mab

IgG3, k

4°C Do Not Freeze

Supplied as a liquid in PBS, pH 7.2.

Partial recombinant corresponding to aa25-124 from human ASAH1 (NP_808592) with GST tag. MW of the GST tag alone is 26kD.

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 ELISA, Western Blot and Immunohistochemistry. Other applications not tested.

Recommended Dilution:
Immunohistochemistry (Formalin fixed paraffin embedded): 3ug/ml
Optimal dilutions to be determined by the researcher.

AA Sequence:
PPWTEDCRKSTYPPSGPTYRGAVPWYTINLDLPPYKRWHELMLDKAPMLKVIVNSLKNMINTFVPSGKVMQVVDEKLPGLLGNFPGPFEEEMKGIAAVTD

Storage and Stability:
May be stored at 4°C. For long-term storage, aliquot and store at 4°C. Do not freeze. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Further dilutions can be made in assay buffer.