Galactosidase, alpha (GLA) (AA 1-289) antibody

Details for Product No. ABIN551188
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Synonyms MGC130872, SMU.877, SCF11.21, AO090005000217, GALA, zgc:101584, Ags, alpha-GAL, AW555139, GM130, mKIAA4150
AA 1-289
(12), (6), (6), (4), (3), (3), (2), (1), (1), (1), (1), (1)
(59), (31), (18), (1), (1), (1)
(63), (6), (2)
(6), (6), (5), (1), (1), (1), (1), (1), (1), (1), (1)
Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)
(54), (44), (15), (13), (11), (10), (10), (4), (3), (3), (3), (2), (2)
Pubmed 4 references available
Quantity 0.1 mL
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Immunogen Recombinant fragment corresponding to a region within amino acids 1 and 289 of Galactosidase alpha
Predicted Reactivity Mouse (80%), Cow (81%)
Characteristics Cellular Localization: Lysosome
Purification Purified by antigen-affinity chromatography..
Alternative Name Alpha-galactosidase A (GLA Antibody Abstract)
Background 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]
Synonyms: GALA antibody, GLA antibody, agalsidase alfa antibody, alpha-gal A antibody, melibiase antibody, alpha-D-galactoside galactohydrolase 1 antibody, alpha-galactosidase A antibody, alpha-D-galactosidase A antibody, "galactosidase, alpha antibody"
Molecular Weight 49 kDa
UniProt P06280

Positive Controls: A431 , H1299 , HeLa , HepG2

Restrictions For Research Use only
Format Liquid
Concentration 1 mg/mL
Buffer 1XPBS, 1%BSA, 20% Glycerol (pH7). 0.01% Thimerosal was added as a preservative.
Preservative Thimerosal (Merthiolate)
Precaution of Use This product contains thimerosal (merthiolate): a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice Avoid multiple freeze-thaw cycles.
Storage -20 °C
Storage Comment Keep as concentrated solution. Aliquot and store at -20°C or below.
Product cited in: Pilon, Schekman: "Protein translocation: how Hsp70 pulls it off." in: Cell, Vol. 97, Issue 6, pp. 679-82, 1999 (PubMed).

Bukau, Horwich: "The Hsp70 and Hsp60 chaperone machines." in: Cell, Vol. 92, Issue 3, pp. 351-66, 1998 (PubMed).

Hang, Fox: "Expression of hsp70 induced in CHO cells by 45.0 degrees C hyperthermia is cell cycle associated and DNA synthesis dependent." in: Cytometry, Vol. 19, Issue 2, pp. 119-25, 1995 (PubMed).

Minota, Cameron, Welch et al.: "Autoantibodies to the constitutive 73-kD member of the hsp70 family of heat shock proteins in systemic lupus erythematosus." in: The Journal of experimental medicine, Vol. 168, Issue 4, pp. 1475-80, 1988 (PubMed).

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Catalog No. ABIN551188
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