Western Blot (WB)
(Western blot analysis of Gla expression in mouse thymus extract (lane 1), mouse liver extract (lane 2) and mouse spleen extract (lane 3). Gla at 46KD was detected using rabbit anti- Gla Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )
Rabbit anti-Mouse Gla Polyclonal Antibody | anti-Gla antibody
Anti-Gla Antibody
Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
Other applications have not been tested.
Western Blot (WB)
(Western blot analysis of Gla expression in mouse thymus extract (lane 1), mouse liver extract (lane 2) and mouse spleen extract (lane 3). Gla at 46KD was detected using rabbit anti- Gla Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )
Western Blot (WB)
(Western blot analysis of Gla expression in mouse thymus extract (lane 1), mouse liver extract (lane 2) and mouse spleen extract (lane 3). Gla at 46KD was detected using rabbit anti- Gla Antigen Affinity purified polyclonal antibody at 0.5ug/mL. The blot was developed using chemiluminescence (ECL) method. )
Background: Alpha-galactosidase is a glycoside hydrolase enzyme that encoded by the GLA gene. This gene is 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.
2. Calhoun DH, Bishop DF, Bernstein HS, Quinn M, Hantzopoulos P, Desnick RJ (1985). "Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A". Proceedings of the National Academy of Sciences of the United States of America. 82 (21): 7364-8.
3. Keating GM (October 2012). "Agalsidase alfa: a review of its use in the management of Fabry disease". BioDrugs. 26 (5): 335-54.
NCBI and Uniprot Product Information
Uniprot Description
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: Carbohydrate Metabolism - galactose; EC 3.2.1.22; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; Lipid Metabolism - glycerolipid; Lipid Metabolism - sphingolipid
Chromosomal Location of Human Ortholog: X E3|X 56.2 cM
Cellular Component: cytoplasm; extracellular region; extracellular space; Golgi apparatus; lysosome
Molecular Function: alpha-galactosidase activity; catalytic activity; galactoside binding; hydrolase activity; protein homodimerization activity; receptor binding
Biological Process: bone mineralization; glycosylceramide catabolic process; negative regulation of nitric oxide biosynthetic process; negative regulation of nitric-oxide synthase activity; oligosaccharide metabolic process
Research Articles on Gla
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Product Notes
The Gla gla (Catalog #AAA178636) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-Gla Antibody reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's Gla can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Western Blot: 0.1-0.5ug/ml. Researchers should empirically determine the suitability of the Gla gla for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Gla, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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