GLA/alpha-Galactosidase A Protein, 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.

Alpha-galactosidase A is responsible for the breakdown of alpha-galactoside in lysosomes and is homologous to human galactosidase (GLA). GLA is involved in the negative regulation of nitric oxide biosynthesis and nitric oxide synthase activity. GLA acts upstream or internally in the catabolic process of glycosylceramide. Mice deficient in alpha-galactosidase A exhibit age-dependent and distinct sensory system deficits. This is a lysosome storage disorder characterized by the accumulation of alpha-galactosylated substrates in tissues. Alpha-galactosidase A is an active target of clinical research: there are currently two treatment options for Fabry disease, recombinant enzyme replacement therapy and drug companion therapy. Tlr-dependent alpha-Gal-A negative regulation is the mechanistic link between pathogen recognition and autolipid antigen induction in NKT cells^[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.

Lyophilized powder

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.