GLA/alpha-Galactosidase A 蛋白, Mouse (HEK293, His)

GLA/alpha-Galactosidase A Protein is responsible for the breakdown of alpha-galactoside in the lysosome. GLA/alpha-Galactosidase A Protein participates in the negative regulation of nitric oxide biosynthesis and nitric oxide synthase activity. A deficiency of GLA/alpha-Galactosidase A Protein causes Fabry disease. GLA/alpha-Galactosidase A Protein, Mouse (HEK293, His) is the recombinant mouse-derived GLA/alpha-Galactosidase A protein, expressed by HEK293, with C-His labeled tag. The total length of GLA/alpha-Galactosidase A Protein, Mouse (HEK293, His) is 388 a.a., with molecular weight of 46-52 kDa.

α-半乳糖苷酶 A 负责溶酶体中 α-半乳糖苷的分解，与人半乳糖苷酶 (GLA) 同源。GLA 参与一氧化氮生物合成和一氧化氮合酶活性的负调节。GLA 在糖基神经酰胺的分解代谢过程中发挥上游或内部作用。缺乏α-半乳糖苷酶A的小鼠表现出年龄依赖性和明显的感觉系统缺陷。这是一种溶酶体贮积症，其特征是组织中α-半乳糖基化底物的积累。α-半乳糖苷酶A是临床研究的活跃靶点：目前法布里病有两种治疗方案，重组酶替代疗法和药物伴随疗法。Tlr 依赖性 α-Gal-A 负调控是 NKT 细胞中病原体识别和自脂抗原诱导之间的机制联系^[1][2][3] [4]。

Measured by its ability to hydrolyze 4-methylumbelliferyl-alpha-D-galactopyranoside. The specific activity is 833.54 pmoL/min/µg, as measured under the described conditions.

Mouse

HEK293

C-His

Q8BGZ6 (L34-R421)

11605  [NCBI]

LDNGLARTPTMGWLHWERFMCNLDCQEEPDACISEQLFMQMAELMVSDGWRDAGYDYLCIDDCWMAPERDSKGRLQADPQRFPSGIKHLANYVHSKGLKLGIYADVGNKTCAGFPGSFGSYDIDAQTFADWGVDLLKFDGCHCDSVVSLENGYKYMALALNRTGRSIVYSCEWPLYLRPFHKPNYTDIQYYCNHWRNFDDVYDSWESIKNILSWTVVYQKEIVEVAGPGSWNDPDMLVIGNFGLSWDQQVTQMALWAIMAAPLLMSNDLRQISSQAKALLQNKDVIAINQDPLGKQGYCFRKENHIEVWERPLSNLAWAVAVRNLQEIGGPCPYTIQISSLGRGLACNPGCIITQLLPEKVHLGFYEWTLTLKTRVNPSGTVLFRLER

46-52 kDa

• 
  Greater than 95% as determined by reducing SDS-PAGE.

无菌粉末

Lyophilized from a 0.2 μm filtered solution of PBS, pH 7.4.

<1 EU/μg, determined by LAL method.

It is not recommended to reconstitute to a concentration less than 100 μg/mL in ddH₂O. For long term storage it is recommended to add a carrier protein (0.1% BSA, 5% HSA, 10% FBS or 5% Trehalose).

Stored at -20°C for 2 years. After reconstitution, it is stable at 4°C for 1 week or -20°C for longer (with carrier protein). It is recommended to freeze aliquots at -20°C or -80°C for extended storage.

Room temperature in continental US; may vary elsewhere.