84-2339-69　［取扱停止］GLA (AGAL_HUMAN, Agalsidase alfa, Alpha D galactosidase A, Alpha D galactoside galactohydrolase 1, Alpha D galactoside galactohydrolase, Alpha gal A, Alpha galactosidase A, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Alpha-galactosidase A, GALA, Galactosidase, alpha, GLA, GLA Protein, Melibiase) 100ul　222953

特徴

• alpha-galactosidase A (alpha-gal A) functions as a lysosomal hydrolase.
• alpha-gal A forms an active homodimer that acts upon a glycolipid substrate, globotriaosylceramide (Gb3).
• The gene encoding alpha-gal A maps to chromosome Xq22.
• Inherited mutations in this gene cause an X-linked recessive glycolipid storage disorder known as Fabry’s disease.
• In Fabry patients, alpha-gal A deficiencies lead to an accumulation of Gb3 in the body.
• The numerous clinical manifestations of the disease include renal and cardiac impairment, severe pain in the extremities and cutaneous lesions known as angiokeratomas.
• Enzyme replacement therapy using recombinant alpha-gal A effectively treats the symptoms of Fabry disease.
• Applications:Suitable for use in Western Blot, Immunohistochemistry.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.