For immunochemical detection of α-galactosidase A
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α-Galactosidase A Rabbit Monoclonal Antibody (Clone 001)

Item No. 38088

Product Insert (PDF)
Technical Information
Synonyms
  • α-D-Galactoside Galactohydrolase
  • Galactosylgalactosylglucosylceramidase
  • GLA
Immunogen
Recombinant human α-galactosidase A
Clone Designation
001
Formulation (Request formulation change)
50 or 100 µl of protein A-affinity purified monoclonal antibody
Storage Buffer
0.2 μm filtered solution in PBS
Host
Rabbit
Isotype
IgG
Applications
ELISA, IP
Cross Reactivity
(+) α-Galactosidase A
Species Reactivity
(+) Human
Shipping & Storage Information
Storage
-80°C
Shipping
Dry ice in continental US; may vary elsewhere
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Certificates of Analysis & Batch Specific Data

Provide batch numbers separated by commas to download or request available product inserts, QC sheets, certificates of analysis, data packs, and GC-MS data.

    50 µl

    $206

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    100 µl

    $329

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    Product Description

    α-Galactosidase A is an acidic hydrolase that catalyzes the removal of terminal α-glucosidase groups from glycoproteins and glycolipids.1,2 It is ubiquitously expressed and functions as a homodimer with each monomer containing an N-terminal a(β/α)8 domain and a C-terminal anti-parallel β domain.3,1 Mutations in GLA, the gene encoding α-galactosidase A, induce lysosomal α-glucosidase A deficiencies, which cause Fabry disease, an X-linked lysosomal storage disorder characterized by hypohidrosis and heat intolerance, as well as cardiac, CNS, and vascular dysfunction.4,2 α-Galactosidase A activity is decreased in dried blood spots from patients with Parkinson's disease but not harboring the Parkinson's disease-associated mutations in LRRK2, LRRK2G2019S, or in glucocerebrosidase.5 Cayman’s α-Galactosidase A Rabbit Monoclonal Antibody (Clone 001) can be used for ELISA and immunoprecipitation (IP) applications.

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Garman, S.C. Structure-function relationships in α-galactosidase A. Acta Paediatr. 96(455), 6-16 (2007).

    2. Dwyer, B., Hu, J., Madduri, A., et al. Affinity purification of human alpha galactosidase utilizing a novel small molecule biomimetic of alpha-D-galactose. Protein Expr. Purif. 177, 105752 (2021).

    3. Fitzmaurice, T.F., Desnick, R.J., and Bishop, D.F. Human α-galactosidase A: High plasma activity expressed by the - 30G—>A allele. J. Inherit. Metab. Dis. 20(5), 643-657 (1997).

    4. Mahmud, H.M. Fabry’s disease — a comprehensive review on pathogenesis, diagnosis and treatment. J. Pak. Med. Assoc. 64(2), 189-194 (2014).

    5. Alcalay, R.N., Wolf, P., Levy, O.A., et al. Alpha galactosidase A activity in Parkinson’s disease. Neurobiol. Dis. 112, 85-90 (2018).