ELISA/assay

Human Acid ceramidase (ASAH1) ELISA Kit

MBS7236851

48-Strip-Wells

470 USD

ELISA Kit

Human Acid ceramidase (ASAH1) ELISA Kit

Acid ceramidase (ASAH1)

acid ceramidase; Acid ceramidase; acid ceramidase; acid CDase; acylsphingosine deacylase; putative 32 kDa heart protein; N-acylsphingosine amidohydrolase (acid ceramidase) 1; Acylsphingosine deacylase; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32

ASAH1

ASAH1; ASAH1; AC; PHP; ASAH; PHP32; ACDase; SMAPME; ASAH; HSD-33; HSD33; AC; ACDase; Acid CDase; PHP32

ASAH1_HUMAN

Human

This assay has high sensitivity and excellent specificity for detection of ASAH-1 No significant cross-reactivity or interference between ASAH-1 and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between ASAH-1 and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C.

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 1.0 ng/mL.

Intended Uses: This ASAH-1 ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human ASAH-1. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

. Principle of the assay: ASAH-1 ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti- ASAH-1 antibody and an ASAH-1-HRP conjugate. The assay sample and buffer are incubated together with ASAH-1-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the ASAH-1 concentration since ASAH-1 from samples and ASAH-1-HRP conjugate compete for the anti- ASAH-1 antibody binding site. Since the number of sites is limited, as more sites are occupied by ASAH-1 from the sample, fewer sites are left to bind ASAH-1-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The ASAH-1 concentration in each sample is interpolated from this standard curve.

1743867

AAC50907.1

44,046 Da

Ceramide Signaling Pathway (138023); Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Signal Transduction Of S1P Receptor Pathway (198819); Sphingolipid Metabolism Pathway (760636); Sphingolipid Metabolism Pathway (82994)

This gene encodes 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. Multiple transcript variants encoding several distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]

ASAH1: Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid. Defects in ASAH1 are the cause of Farber lipogranulomatosis (FL); 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. Defects in ASAH1 are the cause of spinal muscular atrophy with progressive myoclonic epilepsy (SMAPME). An autosomal recessive neuromuscular disorder characterized by childhood onset of motor deficits and progressive myoclonic seizures, after normal developmental milestones. Proximal muscle weakness and generalized muscular atrophy are due to degeneration of spinal motor neurons. Myoclonic epilepsy is generally resistant to conventional therapy. The disease course is progressive and leads to respiratory muscle involvement and severe handicap or early death from respiratory insufficiency. Belongs to the acid ceramidase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Hydrolase; EC 3.5.1.23; Lipid Metabolism - sphingolipid. Chromosomal Location of Human Ortholog: 8p22. Cellular Component: lysosomal lumen. Molecular Function: catalytic activity; ceramidase activity. Biological Process: response to organic substance; sphingolipid metabolic process; glycosphingolipid metabolic process; ceramide metabolic process; lung development. Disease: Farber Lipogranulomatosis

48-Strip-Wells

470 USD

96-Strip-Wells

675