Anti-GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alph

Anti-GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alph
Item number Size Datasheet Manual SDS Delivery time Quantity Price
035832-HRP.200 200 µl - -

3 - 19 business days*

731.00€
 
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to... more
Product information "Anti-GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alph"
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. Applications: Suitable for use in Western Blot, Immunohistochemistry and ELISA. Other applications have not been tested. Recommended Dilutions: Immunohistochemistry: Frozen and formalin fixed and paraffin embedded tissues, Optimal dilutions to be determined by the researcher. Storage and Stability: Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. , , Note: Applications are based on unconjugated antibody.
Keywords: Anti-GAA, EC=3.2.1.20
Supplier: United States Biological
Supplier-Nr: 035832-HRP

Properties

Application: ELISA, IHC, WB
Antibody Type: Polyclonal
Conjugate: HRP
Host: Rabbit
Reactivity: Human
Immunogen: GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of human GAA.
Format: Affinity Purified

Handling & Safety

Storage: -20°C
Shipping: +4°C (International: +4°C)
Caution
Our products are for laboratory research use only: Not for administration to humans!
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