Anti-Acid Ceramidase Antibody | MyBioSource MBS8245649 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Anti-Acid Ceramidase Antibody

catalog :

MBS8245649

quantity :

0.05 mL

price :

175 USD

clonality :

polyclonal

host :

unidentified

conjugate :

nonconjugated

reactivity :

human

application :

western blot

more info or order :

MyBioSource product webpage

image

image 1 :

Western blot analysis of Acid Ceramidase expression in mouse kidney (A), rat heart (B) whole cell lysates.

product information

catalog number :

MBS8245649

products type :

Antibody

products full name :

Anti-Acid Ceramidase Antibody

products short name :

[Acid Ceramidase]

products name syn :

[ASAH; Acid ceramidase; AC; ACDase; Acid CDase; Acylsphingosine deacylase; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32]

other names :

[acid ceramidase isoform c; Acid ceramidase; acid ceramidase; N-acylsphingosine amidohydrolase (acid ceramidase) 1; Acylsphingosine deacylase; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32]

products gene name :

[ASAH1]

other gene names :

[ASAH1; ASAH1; AC; PHP; ASAH; PHP32; ACDase; SMAPME; ASAH; AC; ACDase; Acid CDase; PHP32]

uniprot entry name :

ASAH1_HUMAN

clonality :

Polyclonal

host :

unknown

reactivity :

Human

sequence length :

389

specificity :

Recognizes endogenous levels of Acid Ceramidase protein.

purity :

The antibody was purified by immunogen affinity chromatography.

form :

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

storage stability :

Shipped at 4°C. Upon delivery aliquot and store at -20°C for one year. Avoid freeze/thaw cycles.

tested application :

Western Blot (WB)

app notes :

WB: 1/500 - 1/2000

image1 heading :

Western Blot (WB)

other info1 :

Immunogen: Recombinant full length protein of human Acid Ceramidase

other info2 :

. Source: Rabbit

products description :

Rabbit polyclonal antibody to Acid Ceramidase

ncbi gi num :

189011550

ncbi acc num :

NP_001120977.1

ncbi gb acc num :

NM_001127505.2

uniprot acc num :

Q13510

ncbi pathways :

Ceramide Signaling Pathway (138023); Glycosphingolipid Metabolism Pathway (1270099); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); Signal Transduction Of S1P Receptor Pathway (198819); Sphingolipid Metabolism Pathway (760636); Sphingolipid Metabolism Pathway (82994)

ncbi summary :

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. [provided by RefSeq, Oct 2015]

uniprot summary :

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; Hydrolase; Lipid Metabolism - sphingolipid. Chromosomal Location of Human Ortholog: 8p22. Cellular Component: extracellular space; lysosomal lumen. Molecular Function: catalytic activity; ceramidase activity. Biological Process: ceramide metabolic process; glycosphingolipid metabolic process. Disease: Farber Lipogranulomatosis

size1 :

0.05 mL

price1 :

175 USD

size2 :

0.1 mL

price2 :

220

size3 :

0.2 mL

price3 :

295

more info or order :

MyBioSource product webpage