biological source
rabbit
conjugate
unconjugated
material
colorless
clone
5L2L10, monoclonal
form
liquid
species reactivity
rat, rat, human
concentration
0.8 mg/mL
technique(s)
western blot: 1:500 - 1:2000
color
colorless
isotype
IgG
immunogen sequence
QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... GAA(2548)
Application
WB
General description
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.
Immunogen
A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
存储类别
10 - Combustible liquids
wgk
WGK 1
法规信息
常规特殊物品
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