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SAB5702125

Sigma-Aldrich

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

Synonym(s):

GAA, LYAG, glucosidase alpha, acid

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.46

biological source

rabbit

Quality Level

100

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.

P10253

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... GAA(2548)

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.

Application

WB

wgk_germany

WGK 1

Certificates of Analysis (COA)

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