быстрая доставка ~4 недели
Размер
- 100 μL
- 200 μL
- 50 μL
GAA Rabbit mAb (A19234)
- Описание
- Характеристики
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Overview
Product name GAA Rabbit mAb Catalog No. A19234 Host species Rabbit Purification method Affinity purification Isotype IgG CloneNo. ARC2392 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.Immunogen information
Immunogen A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253). Sequence QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV Gene ID Swiss Prot Synonyms LYAG Calculated MW 105kDa Observed MW 76kDa/105kDa Applications
Reactivity Human Tested applications WB Recommended dilution - WB 1:500 - 1:1000
Storage buffer Store at -20℃. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.Key application Western blotting Positive samples 293T, HepG2, DU145 Cellular location Lysosome, Lysosome membrane 
Western blot analysis of extracts of various cell lines, using GAA antibody (A19234) at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution.
Lysates/proteins: 25μg per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit (RM00020).
Exposure time: 10s. -
Key applications Western blotting Host species Кролик Antibody type Primary Antibodies Reactivity Человек Immunogen A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253). Isotype IgG