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Recombinant Human Acid ceramidase (ASAH1) is a partial length protein expressed with N-terminal 6xHis-SUMO-tagged in the E.coli. Its expression region corresponds to 22-395aa of human ASAH1 protein. Its purity was determined by SDS-PAGE and reached up to 90% and presented a molecular mass band of 58.7 kDa on the gel. This recombinant ASAH1 protein may be used to synthesize antibodies against ASAH1. ASAH1 proteins in-stock are available. ASAH1 is lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH. Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation. This enzyme is overexpressed in multiple human cancers and may play a role in cancer progression. ASAH1 mutations are associated with the lysosomal storage disorder, Farber lipogranulomatosis, and a neuromuscular disorder, spinal muscular atrophy with progressive myoclonic epilepsy.
Recombinant Human Acid ceramidase (ASAH1) is a partial length protein expressed with N-terminal 6xHis-SUMO-tagged in the E.coli. Its expression region corresponds to 22-395aa of human ASAH1 protein. Its purity was determined by SDS-PAGE and reached up to 90% and presented a molecular mass band of 58.7 kDa on the gel. This recombinant ASAH1 protein may be used to synthesize antibodies against ASAH1. ASAH1 proteins in-stock are available.
ASAH1 is lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH. Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation. This enzyme is overexpressed in multiple human cancers and may play a role in cancer progression. ASAH1 mutations are associated with the lysosomal storage disorder, Farber lipogranulomatosis, and a neuromuscular disorder, spinal muscular atrophy with progressive myoclonic epilepsy.
| Cat.No | ACP02381 | Target Name | ASAH1 |
|---|---|---|---|
| Form | Liquid or Lyophilized powder | Expression System | E.coli |
| Expression Range | 22-395aa | Mol Weight | 58.7kDa |
| Protein Length | Full Length of Mature Protein | Purity | Greater than 90% as determined by SDS-PAGE. |
| Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | Q13510 |
|---|
Uniprot Id
Q13510
Target Species
Human
Target Name
ASAH1
Target Full Name
Acid ceramidase
Target Function
Lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH. Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation. Has a higher catalytic efficiency towards C12-ceramides versus other ceramides. Also catalyzes the reverse reaction allowing the synthesis of ceramides from fatty acids and sphingosine. For the reverse synthetic reaction, the natural sphingosine D-erythro isomer is more efficiently utilized as a substrate compared to D-erythro-dihydrosphingosine and D-erythro-phytosphingosine, while the fatty acids with chain lengths of 12 or 14 carbons are the most efficiently used. Has also an N-acylethanolamine hydrolase activity. By regulating the levels of ceramides, sphingosine and sphingosine-1-phosphate in the epidermis, mediates the calcium-induced differentiation of epidermal keratinocytes. Also indirectly regulates tumor necrosis factor/TNF-induced apoptosis. By regulating the intracellular balance between ceramides and sphingosine, in adrenocortical cells, probably also acts as a regulator of steroidogenesis.; May directly regulate steroidogenesis by binding the nuclear receptor NR5A1 and negatively regulating its transcriptional activity.
Target Involvement
Farber lipogranulomatosis (FRBRL); Spinal muscular atrophy with progressive myoclonic epilepsy (SMAPME)
Target Subcellular Location
Lysosome. Secreted.; [Isoform 2]: Nucleus. Cytoplasm.
Target Protein Families
Acid ceramidase family
Target Tissue Specificity
Broadly expressed with higher expression in heart.
Target Research Area
Signal Transduction
Target Synonyms
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; N-acylsphingosine deacylase; PHP; PHP32; Putative 32 kDa heart protein; SMAPME
Target Background
This gene encodes a member of the acid ceramidase family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. Processing of this preproprotein generates alpha and beta subunits that heterodimerize to form the mature lysosomal enzyme, which catalyzes the degradation of ceramide into sphingosine and free fatty acid. This enzyme is overexpressed in multiple human cancers and may play a role in cancer progression. Mutations in this gene are associated with the lysosomal storage disorder, Farber lipogranulomatosis, and a neuromuscular disorder, spinal muscular atrophy with progressive myoclonic epilepsy.
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