Recombinant Galactosylceramidase (GALC) | MyBioSource MBS2009657 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Galactosylceramidase (GALC)

catalog :

MBS2009657

quantity :

0.01 mg

price :

205 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS2009657

products type :

Recombinant Protein

products full name :

Recombinant Galactosylceramidase (GALC)

products short name :

Galactosylceramidase (GALC)

other names :

galactocerebrosidase isoform a; Galactocerebrosidase; galactocerebrosidase; GALCERase; galactosylceraminidase; galactocerebroside beta-galactosidase; galactosylceramide beta-galactosidase; galactosylceramidase; Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase

products gene name :

GALC

other gene names :

GALC; GALC; GALCERase

uniprot entry name :

GALC_HUMAN

host :

E Coli

sequence length :

685

sequence :

The sequence of the target protein is listed below. DGLGNL TIIIETMSHK HSKCIRPFLP YFNVSQQFAT FVLKGSFSEI PELQVWYTKL GKTSERFLFK QLDSLWLLDS DGSFTLSLHE DELFTLTTLT TGRKGSYPLP PKSQPFPSTY KDDFNVDYPF FSEAPNFADQ TGVFEYFTNI EDPGEHHFTL RQVLN

purity :

> 95%

form :

Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.

storage stability :

Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

tested application :

SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP). May be suitable for use in other assays to be determined by the end user.

other info1 :

Organism: Homo sapiens (Human). Expression System: Prokaryotic expression. Residues: Asp375~Asn535 (Accession # P54803) with N-terminal His-Tag. Predicted isoelectric point: 5.7

other info2 :

Endotoxin Level: <1.0EU per 1ug (determined by the LAL method). Reconstitution: Reconstitute in sterile PBS, pH7.2-pH7.4.

products description :

About the Marker: Effective Size Range: 10kDa to 70kDa. Protein bands: 10kDa, 14kDa, 18kDa, 22kDa, 26kDa, 33kDa, 44kDa and70kDa. Double intensity bands: The 26kDa, 18kDa, 10kDa bands are at doubleintensity to make location and size approximation of proteins of interestquick and easy. Ready-to-use: No need to heat, dilute or add reducing agents before use.

ncbi gi num :

83281450

ncbi acc num :

NP_000144.2

ncbi gb acc num :

NM_000153.3

uniprot acc num :

P54803

ncbi mol weight :

20.1kDa

ncbi pathways :

Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Sphingolipid Metabolism Pathway (82994); Sphingolipid Metabolism Pathway (119543); Sphingolipid Metabolism Pathway (369)

ncbi summary :

This gene encodes a lysosomal protein which hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Mutations in this gene have been associated with Krabbe disease, also known as globoid cell leukodystrophy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008]

uniprot summary :

GALC: Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. Defects in GALC are the cause of leukodystrophy globoid cell (GLD); also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified. Belongs to the glycosyl hydrolase 59 family. 4 isoforms of the human protein are produced by alternative splicing. Protein type: EC 3.2.1.46; Hydrolase; Lipid Metabolism - sphingolipid. Chromosomal Location of Human Ortholog: 14q31. Cellular Component: lysosomal lumen; lysosome. Molecular Function: galactosylceramidase activity. Biological Process: sphingolipid metabolic process; carbohydrate metabolic process; glycosphingolipid metabolic process; galactosylceramide catabolic process. Disease: Krabbe Disease

size1 :

0.01 mg

price1 :

205 USD

size2 :

0.05 mg

price2 :

460

size3 :

0.1 mg

price3 :

715

more info or order :

MyBioSource product webpage