α-gal A Antibody (C6): sc-517442

α-gal A Antibody (C6) is a mouse monoclonal IgG2b kappa light chain antibody that detects alpha-gal A protein of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). Alpha-gal A (C6) antibody is available as a non-conjugated antibody. Alpha-galactosidase A (α-gal A) protein plays a crucial role as a lysosomal hydrolase, functioning to break down glycolipids, specifically globotriaosylceramide (Gb3). This enzymatic activity is vital for maintaining cellular homeostasis, as Gb3 accumulation due to α-gal A deficiencies can lead to serious health issues, including Fabry disease, an X-linked recessive disorder characterized by glycolipid buildup in various tissues. In patients with Fabry disease, lack of functional α-gal A results in renal and cardiac complications, severe pain in the extremities, and distinctive skin lesions known as angiokeratomas. Enzyme replacement therapy using recombinant α-gal A has proven effective in alleviating symptoms associated with this debilitating condition, highlighting α-gal A′s significance in therapeutic interventions.

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α-gal A Antibody (C6) References:

1. Infusion of alpha-galactosidase A reduces tissue globotriaosylceramide storage in patients with Fabry disease.  |  Schiffmann, R., et al. 2000. Proc Natl Acad Sci U S A. 97: 365-70. PMID: 10618424
2. Fabry disease: preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice.  |  Ioannou, YA., et al. 2001. Am J Hum Genet. 68: 14-25. PMID: 11115376
3. A phase 1/2 clinical trial of enzyme replacement in fabry disease: pharmacokinetic, substrate clearance, and safety studies.  |  Eng, CM., et al. 2001. Am J Hum Genet. 68: 711-22. PMID: 11179018
4. Fabry disease: diagnosis and treatment.  |  Breunig, F., et al. 2003. Kidney Int Suppl. S181-5. PMID: 12694340
5. A pharmacogenetic approach to identify mutant forms of α-galactosidase A that respond to a pharmacological chaperone for Fabry disease.  |  Wu, X., et al. 2011. Hum Mutat. 32: 965-77. PMID: 21598360
6. Functional characterisation of alpha-galactosidase a mutations as a basis for a new classification system in fabry disease.  |  Lukas, J., et al. 2013. PLoS Genet. 9: e1003632. PMID: 23935525
7. Functional and Clinical Consequences of Novel α-Galactosidase A Mutations in Fabry Disease.  |  Lukas, J., et al. 2016. Hum Mutat. 37: 43-51. PMID: 26415523
8. α-Galactosidase A Genotype N215S Induces a Specific Cardiac Variant of Fabry Disease.  |  Oder, D., et al. 2017. Circ Cardiovasc Genet. 10: PMID: 29018006
9. Alpha galactosidase A activity in Parkinson's disease.  |  Alcalay, RN., et al. 2018. Neurobiol Dis. 112: 85-90. PMID: 29369793
10. α-Galactosidase A/lysoGb3 ratio as a potential marker for Fabry disease in females.  |  Baydakova, GV., et al. 2020. Clin Chim Acta. 501: 27-32. PMID: 31770509
11. Fabry's disease: alpha-galactosidase deficiency.  |  Kint, JA. 1970. Science. 167: 1268-9. PMID: 5411915
12. Physical mapping in the region of the Bruton's tyrosine kinase and alpha-galactosidase A gene loci in proximal Xq22.  |  Sweatman, AK., et al. 1994. Hum Genet. 94: 624-8. PMID: 7989038

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-α-gal A Antibody (C6) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-α-gal A Antibody (C6) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

α-gal A Antibody (C6) Specifications

Alternate Product Name: α-gal A

Product Clone Name: C6

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2b kappa light chain

Amino Acids: 81-429

Epitope Target: α-gal A

Epitope Species: human

Product Reactivity/Detection: α-gal A

Species Reactivity/Detection: human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, IHC(P), ELISA

Vialing Information: Each vial contains 100 µg IgG_2b kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide, 0.1% gelatin and 1% glycerol

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for α-gal A Antibody (C6): N/A