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Product Name ASAH1 Chinese Name 酸性神经酰胺酶1抗体 Alias AC; ACDase; Acid CDase; Acid ceramidase; Acid ceramidase precursor; Acid ceramidase subunit beta; Acylsphingosine deacylase; ASAH 1; ASAH; ASAH1; ASAH1_HUMAN; FLJ21558; FLJ22079; N acylsphingosine amidohydrolase (acid ceramidase) 1; N acylsphingosine amidohydrolase 1; N acylsphingosine amidohydrolase; N-acylsphingosine amidohydrolase; PHP; PHP32; Putative 32 kDa heart protein. Research Area Tumour Cardiovascular Cell biology Neurobiology Signal transduction The new supersedes the old Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, ) Applications ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 29kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Acid ceramidase subunit beta: 301-395/395 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Acid ceramidase catalyzes the degradation of ceramide in normal tissues, and deficiency leads to accumulation of ceramide in tissues, a hallmark of Farber disease. Effected individuals experience early onset joint problems and neurological problems, owing to mutations in the acid ceramidase gene. Bioinformatic analysis of gene expression also reveals acid ceramidase to be among the 5 most important genes associated with melanoma. In addition to ceramide hydrolysis, purified acid ceramidase also exhibits the ability to catalyze ceramide synthesis, utilizing [14C]lauric acid and sphingosine as substrates. Interestingly, pH regulates which reaction is favored; for hydrolysis the pH optimum is 4.5, whereas for the reverse reaction favors a pH of 5.5, further supporting a complex and central role for acid ceramidase in sphingolipid metabolism.
Function:
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid.
Subunit:
Heterodimer of one alpha and one beta subunit.
Subcellular Location:
Lysosome.
Tissue Specificity:
Broadly expressed with highest expression in heart.
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.
Similarity:
Belongs to the acid ceramidase family.
SWISS:
Q13510
Gene ID:
427
Database links:Entrez Gene: 427 Human
Entrez Gene: 11886 Mouse
Omim: 613468 Human
SwissProt: Q13510 Human
SwissProt: Q9WV54 Mouse
Product Picture Tissue/cell: Mouse stomach tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-ASAH1 Polyclonal Antibody, Unconjugated(SL12976R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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