GAA (NM_001079804) Human Mass Spec Standard

CAT#: PH308033

GAA MS Standard C13 and N15-labeled recombinant protein (NP_001073272)

  View other "GAA" proteins (14)

USD 2,055.00

3 Weeks*

Size
    • 10 ug

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Specifications

Product Data
Tag C-Myc/DDK
Species Human
Expression Host HEK293
Expression cDNA Clone or AA Sequence RC208033
Predicted MW 105.3 kDa
Protein Sequence
Purity > 80% as determined by SDS-PAGE and Coomassie blue staining
Concentration 50 ug/ml as determined by BCA
Labeling Method Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine
Buffer 100 mM glycine, 25 mM Tris-HCl, pH 7.3. Store at -80°C. Avoid repeated freeze-thaw cycles. Stable for 3 months from receipt of products under proper storage and handling conditions.
Reference Data
RefSeq NP_001073272
RefSeq Size 3517
RefSeq ORF 2856
Synonyms LYAG
Locus ID 2548
UniProt ID P10253
Cytogenetics 17q25.3
Summary '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]'
Protein Families Druggable Genome, Transmembrane
Protein Pathways Galactose metabolism, Lysosome, Metabolic pathways, Starch and sucrose metabolism

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*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.