Recombinant N-Acylsphingosine Amidohydrolase 1 (ASAH1) | MyBioSource MBS2030445 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant N-Acylsphingosine Amidohydrolase 1 (ASAH1)

catalog :

MBS2030445

quantity :

0.01 mg

price :

140 USD

more info or order :

MyBioSource product webpage

image

image 1 :

product information

catalog number :

MBS2030445

products type :

Recombinant Protein

products full name :

Recombinant N-Acylsphingosine Amidohydrolase 1 (ASAH1)

products short name :

[N-Acylsphingosine Amidohydrolase 1]

other names :

[N-acylsphingosine amidohydrolase 1, partial; 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

host :

E.coli

reactivity :

Human

purity :

> 97%

form :

100mM NaHCO3, 500mM NaCl, pH8.3, containing 1mM DTT, 0.01% SKL, 5% Trehalose and Proclin300.

concentration :

Original Concentration: 200ug/mL

storage stability :

Storage: Avoid repeated freeze/thaw cycles. Store at 2-8ºC for one month. Aliquot and store at -80ºC for 12 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

tested application :

Positive Control, Immunogen, SDS-PAGE, WB. (May be suitable for use in other assays to be determined by the end user.)

image1 heading :

SDS-Page

other info1 :

Source: Prokaryotic expression. Residues: Cys143~Trp395. Tags: N-terminal His Tag. Subcellular Location: Lysosome. Traits: Freeze-dried powder

other info2 :

Predicted isoelectric point: 7.5. Predicted Molecular Mass: 32.7kDa. Accurate Molecular Mass: 33kDa as determined by SDS-PAGE reducing conditions. Usage: Reconstitute in 100mM NaHCO3, 500mM NaCl (pH8.3) to a concentration of 0.1-1.0 mg/mL. Do not vortex.

ncbi gi num :

169403914

ncbi acc num :

BAG12377.1

ncbi mol weight :

44,046 Da

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 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]

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: Lipid Metabolism - sphingolipid; EC 3.5.1.23; Hydrolase. 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

size1 :

0.01 mg

price1 :

140 USD

size2 :

0.05 mg

price2 :

255

size3 :

0.1 mg

price3 :

390

size4 :

0.2 mg

price4 :

480

size5 :

0.5 mg

price5 :

935

size6 :

1 mg

price6 :

1380

more info or order :

MyBioSource product webpage