Specifications
- Pack type:Vial
- Conjugation:Unconjugated
- Protein/Peptide Type:Recombinant
- Source:E. coli
- Species:Human
- Size:50 µg
- Tag sequence:MGHHHHHHSGSEF
- Storage Conditions:−20 °C
- Endotoxin Content:<1.0 EU per 1 µg (determined by the LAL method)
- Gene ID:427
- Reconstitution Instructions:Reconstitute in 10 mM PBS (pH 7.4) to a concentration of 0.1 - 1.0 mg/ml. Do not vortex.
- Endotoxin-free:N
- Carrier-Free:Y
- Protease-free:N
- Animal-Free:Y
- Protein Synonyms:N-Acylsphingosine Amidohydrolase 1
- UniProtKB:Q13510
- Protein/Peptide Name:ASAH1 (prokaryotic)
- Purity:97 - 100%
- Molecular Weight:33 kDa
- Sequence:Thr144~Trp395
- Endotoxin Level:Low
- Concentration:0.2 mg/ml
- Formulation:Lyophilized from PBS, pH 7.4, containing 0.01% SKL, 5% Trehalose
- Nuclease-free:N
- Shipping Temperature:4 °C
- Tested Applications:Positive control, Immunogen, SDS-PAGE, Western blot.
- Cat. No.:MSPP-RPC302HU1
Specifications
About this item
This is a ASAH1 recombinant protein (prokaryotic), Human is sequencing from Thr144~Trp395 with 97 to 100% purity. Lyophilized from PBS, pH 7.4, containing 0.01% SKL, 5% Trehalose with 0.2 mg/ml.
- High quality, purity, reproducibility and effectiveness
- Offers customized buffers and tag options
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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.