Recombinant Mouse Galactocerebrosidase (Galc)

中文名稱：

小鼠Galc重組蛋白
貨號：

CSB-YP009196MO
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

小鼠Galc重組蛋白
貨號：

CSB-EP009196MO
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

小鼠Galc重組蛋白
貨號：

CSB-EP009196MO-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

小鼠Galc重組蛋白
貨號：

CSB-BP009196MO
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

Galc
Uniprot No.：

P54818
別名：

Galc; Galactocerebrosidase; GALCERase; Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase
種屬：

Mus musculus (Mouse)
蛋白長度：

Full Length of Mature Protein
表達區域：

43-684
氨基酸序列

YVLDDSDG LGREFDGIGA VSGGGATSRL LVNYPEPYRS EILDYLFKPN FGASLHILKV EIGGDGQTTD GTEPSHMHYE LDENYFRGYE WWLMKEAKKR NPDIILMGLP WSFPGWLGKG FSWPYVNLQL TAYYVVRWIL GAKHYHDLDI DYIGIWNERP FDANYIKELR KMLDYQGLQR VRIIASDNLW EPISSSLLLD QELWKVVDVI GAHYPGTYTV WNAKMSGKKL WSSEDFSTIN SNVGAGCWSR ILNQNYINGN MTSTIAWNLV ASYYEELPYG RSGLMTAQEP WSGHYVVASP IWVSAHTTQF TQPGWYYLKT VGHLEKGGSY VALTDGLGNL TIIIETMSHQ HSMCIRPYLP YYNVSHQLAT FTLKGSLREI QELQVWYTKL GTPQQRLHFK QLDTLWLLDG SGSFTLELEE DEIFTLTTLT TGRKGSYPPP PSSKPFPTNY KDDFNVEYPL FSEAPNFADQ TGVFEYYMNN EDREHRFTLR QVLNQRPITW AADASSTISV IGDHHWTNMT VQCDVYIETP RSGGVFIAGR VNKGGILIRS ATGVFFWIFA NGSYRVTADL GGWITYASGH ADVTAKRWYT LTLGIKGYFA FGMLNGTILW KNVRVKYPGH GWAAIGTHTF EFAQFDNFRV EAAR
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 668
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

功能：

Hydrolyzes the galactose ester bonds of glycolipids such as galactosylceramide and galactosylsphingosine. 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.
基因功能參考文獻：
1. Data show that transgenic galactocerebrosidase (GALC) activity was mainly localized at the Purkinje cells layer in the cerebellum of the AAV-treated twitcher mice. PMID: 26115766
2. The results of this study indicated that the greater neurochemical pathology observed in the optic nerve than in the sciatic nerve of beta-gal -/- mice is likely due to the greater glycolipid storage in optic nerve. PMID: 25694553
3. This study demonistrated that galactosylceramidase Deficiency also casue neuromuscular dysfunction. PMID: 25632136
4. results show that GALCtwi-5J, a spontaneous mutation in murine GALC precisely matches the E130K missense mutation in patients with infantile Krabbe disease PMID: 23620143
5. Insights into the mechanisms underlying galactosylceramidase regulation of early post-natal neurogenic niches improve our understanding of the multi-component pathology of globoid cell leukodystrophy . PMID: 22859505
6. The crystal structures of GALC and the GALC-product complex, revealing a novel domain architecture with a previously uncharacterized lectin domain not observed in other hydrolases, are presented. PMID: 21876145
7. Data show that GALC and, possibly, other enzymes for the maintenance of niche functionality and health tightly control the concentration of these sphingolipids within HSPCs. PMID: 20511539
8. GALC is not restricted to myelinating cells but also to several neuronal cell types in the nervous system, such as hippocampal pyramidal neurons and cerebellar neurons. PMID: 15248301
9. Direct administration of these viral particles into the brains of neonatal mice with globoid cell leukodystrophy resulted in sustained expression of GALC activity, improved myelination PMID: 15851012
10. mutant oligodendrocytes can internalize exogenous galactocerebrosidase and maintain stable myelin, demonstrating that exogenous enzyme replacement will be a key strategy in the therapy of globoid cell leukodystrophy PMID: 16352725
11. Lentiviral vectors were designed and optimized for transfer of Galc expression in Twitcher brain. PMID: 16732552
12. Mouse model of globoid cell leukodystrophy contains a premature stop codon (W339X) in the galactosylceramidase (GALC) gene that abolishes enzymatic activity. PMID: 16759875
13. These results suggest that GALC deficiency not only affects myelinating glia but also leads to neuronal dysfunction. The contemporaneous neuropathology might help to explain the limited efficacy of current gene and cell therapies. PMID: 19185028
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相關疾病：

Defects in Galc are the cause of the 'twitcher' phenotype; an autosomal recessive leukodystrophy similar to the human disease (Krabbe disease). This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin.
亞細胞定位：

Lysosome.
蛋白家族：

Glycosyl hydrolase 59 family
組織特異性：

Detected in brain and kidney.
數據庫鏈接：

KEGG: mmu:14420

STRING: 10090.ENSMUSP00000021390

UniGene: Mm.5120