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

Details for Product No. ABIN551188
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Antigen
Synonyms MGC130872, SMU.877, SCF11.21, AO090005000217, GALA, zgc:101584, Ags, alpha-GAL, AW555139, GM130, mKIAA4150
Epitope
AA 1-289
(11), (8), (6), (6), (3), (3), (2), (1), (1), (1)
Reactivity
Human
(65), (26), (17), (1), (1), (1)
Host
Rabbit
(60), (11), (2)
Clonality
Polyclonal
Conjugate
Un-conjugated
(8), (8), (7), (2), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)
(60), (42), (26), (15), (9), (9), (7), (6), (6), (4), (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
Comment

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).

Validation Images
Catalog No. ABIN551188
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