Product info:

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.

Alternative names:

GAA; LYAG

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

Lysosome, Lysosome membrane

Isotype:

IgG

Immunogen:

A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).

Positive control:

DU145, Mouse liver, Mouse ovary, Mouse brain, Rat brain

AA Sequence:

VVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSG

Purification:

Affinity purification

Molecular weight:

Calculated MW: 105kDa Observed MW: 105KDa

Form:

Liquid

Applications:

Request info at info@sobekbio.com

Dilution:

WB 1:500 - 1:2000 IF 1:50 - 1:200 IP 1:50 - 1:200

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

More info:

Email: info@sobekbio.com

Orders:

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