AGLU
Reactivity: Human
WB
Host: Mouse
Monoclonal
43G7
unconjugated
Application Notes
Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Format
Liquid
Concentration
0.25 mg/mL
Buffer
PBS containing 0.09 % (W/V) Sodium Azide as preservative
Preservative
Sodium azide
Precaution of Use
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice
Avoid repeated freezing and thawing.
Storage
4 °C/-20 °C
Storage Comment
Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Target
AGLU
(alpha-Glucosidase (AGLU))
Alternative Name
alpha-Glucosidase
Background
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.Synonyms: Acid maltase, Aglucosidase alfa, GAA, LYAG, Lysosomal alpha-glucosidase