Acid Ceramidase 1, Polyclonal Antibody

N-acylsphingosine amidohydrolase (Acid ceramidase) 1; ASAH1

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 8; NC_000008.10 (17913925..17942507, complement). Location: 8p22

Polyclonal

IgG

Rabbit

Antigen Immunoaffiinity Purification

Provided as solution in phosphate buffered saline with 0.08% sodium azide

Product should be stored at -20 degree C. Aliquot to avoid freeze/thaw cycles

Small volumes of anti-ASAH1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Acid ceramidase is a lipid hydrolyase responsible for the degradation of ceramide into sphingosine and free fatty acids within lysosomes. It can also synthesize ceramide from sphingosine and free fatty acids as well. The reverse activity is pH dependent (6.0 vs 4.5, respectively). This suggests that the enzyme may have diverse functions within cells dependent on its subcellular location and the local pH. Recent studies have shown that acid ceramidase activity is aberrantly expressed in several human cancers and that it might be a useful drug target. As an example, inhibitors of enzyme activity have been shown to slow the growth of cancer cells alone or in combination with other established, anti-oncogenic treatments. Aberrant activity has also been shown in Alzheimer?s disease and overexpression may prevent insulin resistant (Type II) diabetes by free fatty acids.

Antigen Immunoaffinity Purified Polyclonal

Western Blot (WB), ELISA (EIA)

Immunohistochemical staining of human heart tissue using Acid Ceramidase 1 antibody at 10 ug/ml.

44 kDa[Similar Products]

N-acylsphingosine amidohydrolase (acid ceramidase) 1

acid ceramidase; acid CDase; acylsphingosine deacylase; putative 32 kDa heart protein

N-acylsphingosine amidohydrolase (Acid ceramidase) 1 preproprotein isoform a variant

Q53H01_HUMAN

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: EC 3.5.1.23; Lipid Metabolism - sphingolipid; Hydrolase

Cellular Component: lysosome

Molecular Function: hydrolase activity; ceramidase activity

Biological Process: response to organic substance; lipid metabolic process; lung development

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