Human ASAH1 ELISA Kit
Supplier: CLOUD-CLONE CORP MS
This assay has high sensitivity and excellent specificity for detecting Human ASAH1 (N-Acylsphingosine Amidohydrolase 1). The assay range is from 1.56 to 100 ng/ml (Sandwich kit) with a sensitivity of 0.57 ng/ml. There is no detectable cross-reactivity with other relevant proteins. Activity loss rate and accelerated stability test ect have been conducted to guarantee the best performance of the products after long storage and delivery.
- High sensitivity and specificity
- Perfect reproducibility and consistency across batches
- Quality control with three-level inspections
- Wide range of targets/species available
- Intra-assay: CV<10%; Inter-assay: CV<12%
Acid ceramidase is a heterodimeric protein consisting of a nonglycosylated alpha subunit and a glycosylated beta subunit that is cleaved to the mature enzyme posttranslationally. The encoded protein catalyzes the synthesis and degradation of ceramide into sphingosine and fatty acid. Mutations in this gene have been associated with a lysosomal storage disorder known as Farber disease. Two transcript variants encoding distinct isoforms have been identified for this gene. In melanocytic cells ASAH1 gene expression may be regulated by MITF. Defects in ASAH1 are the cause of 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.
Caution: For research use only. Not for use in clinical diagnostic procedures. Please proper stored each component based on the instruction.
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