蛋白

重组半乳糖苷酶甲(GLa)

MBS2010065

0.01毫克

200美元

重组蛋白

重组半乳糖苷酶甲(GLa)

半乳糖苷酶甲(GLa)

alpha-galactosidase A; Alpha-galactosidase A; alpha-galactosidase A; melibiase; alpha-gal A; agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; galactosidase, alpha; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase

GLa

GLA;GLA;GALA

AGAL_HUMAN

大肠杆菌

429

The sequence of the target protein is listed below. GSFG YYDIDAQTFA DWGVDLLKFD GCYCDSLENL ADGYKHMSLA LNRTGRSIVY SCEWPLYMWP FQKPNYTEIR QYCNHWRNFA DIDDSWKSIK SILDWTSFNQ ERIVDVAGPG GWNDPDMLVI GNFGLSWNQQ VTQMALWAIM AAPLFMSNDL RHISPQAKAL LQDKDVIAIN QDPLGKQGYQ LRQGDNFEVW ERPLSGLAWA VAMINRQEIG GPRSYTIAVA S

> 95%

Supplied as lyophilized form in PBS, pH7.4, containing 1mM DTT, 5% trehalose, 0.01% sarcosyl and preservative.

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.

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

Organism: Homo sapiens (Human). Expression System: Prokaryotic expression. Residues: Gly147~Ser371 (Accession # P06280) with two N-terminal Tags, His-tag and S-tag. Predicted isoelectric point: 5.1

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

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.

4504009

NP_000160.1

NM_000169.2

P06280

31.4kDa

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Glycerolipid Metabolism Pathway (82986); Glycerolipid Metabolism Pathway (361); Glycosphingolipid Biosynthesis - Globo Series Pathway (82996); Glycosphingolipid Biosynthesis - Globo Series Pathway (371); Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135)

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008]

GLA: Defects in GLA are the cause of Fabry disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family. Protein type: Glycan Metabolism - glycosphingolipid biosynthesis - globo series; EC 3.2.1.22; Lipid Metabolism - sphingolipid; Carbohydrate Metabolism - galactose; Hydrolase; Lipid Metabolism - glycerolipid. Chromosomal Location of Human Ortholog: Xq22. Cellular Component: Golgi apparatus; lysosomal lumen; lysosome; cytoplasm; extracellular region. Molecular Function: protein binding; protein homodimerization activity; hydrolase activity; alpha-galactosidase activity; galactoside binding; catalytic activity; receptor binding. Biological Process: sphingolipid metabolic process; glycoside catabolic process; negative regulation of nitric-oxide synthase activity; negative regulation of nitric oxide biosynthetic process; glycosphingolipid catabolic process; glycosylceramide catabolic process; glycosphingolipid metabolic process; oligosaccharide metabolic process. Disease: Fabry Disease

0.01毫克

200美元

0.05毫克

430

0.1毫克

665