GAA Rabbit Monoclonal Antibody
Product Specifications
Background
This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]
Product Name Alternative
LYAG
Gene Name
GAA
Gene ID
2548
Swiss Prot
P10253
Host
Rabbit
Reactivity
Human, Mouse, Rat
Clonality
Monoclonal
Conjugation
Unconjugated
Applications
WB
Purification
Affinity Purification
Dilution
WB 1:1000-1:5000
Form
Liquid
Buffer
Supplied in 50mM Tris-Glycine (pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.
Modification
Unmodified
Molecular Weight
Calculated MW:105 kDa; Observed MW:76 kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Isotype
IgG
Available Sizes
Frequently Asked Questions
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