GAA Recombinant Rabbit mAb
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]
CAS Number
9000-83-3
Structure Composition
Store at -20°C. 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.
Product Name Alternative
LYAG
Swiss Prot
P10253
Reactivity
Human, Mouse, Rat
Immunogen
A synthetic peptide of human GAA
Conjugation
Unconjugated
Applications
WB
Dilution
WB: 1:1000-1:5000
Purity
Affinity Purification
Modification
Unmodification
Molecular Weight
76 kDa
Storage Conditions
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Notes
For research use only, not for use in diagnostic procedure.
Specificity
IgG
Applications Notes
Western blot analysis of extracts from 293T cells and Human placenta tissue using BS45947 at 1: 1000.
Host or Source
Rabbit
Available Sizes
Frequently Asked Questions
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