catalog number :
MBS455608
products type :
ELISA Kit
products full name :
Human Glucosidase Alpha, Acid (GaA) ELISA Kit
products short name :
[Glucosidase Alpha, Acid (GaA)]
products name syn :
[LYAG; Acid Alpha-Glucosidase; Lysosomal Alpha-Glucosidase; Pompe Disease Glycogen Storage Disease Type II; Acid Maltase; Aglucosidase Alfa]
other names :
[lysosomal alpha-glucosidase preproprotein; Lysosomal alpha-glucosidase; lysosomal alpha-glucosidase; glucosidase alpha, acid; Acid maltase; Aglucosidase alfa]
products gene name :
[GaA]
other gene names :
[GAA; GAA; LYAG]
storage stability :
For unopened kit, all reagents should be kept according to the labels on vials. The TMB Substrate, Wash Buffer, Stop Solution should be stored at 4 degree C. All others should be stored at -20 degree C.
image1 heading :
Typical Testing Data/Standard Curve (for reference only)
other info1 :
Samples: Serum, plasma, tissue homogenates, cell lysates and other biological fluids. Assay Type: Sandwich. Detection Range: 0.156-10ng/mL. Sensitivity: 0.064ng/mL
other info2 :
Intra Assay: CV<10%. Inter Assay: CV<12%
ncbi acc num :
NP_000143.2
ncbi gb acc num :
NM_000152.4
ncbi mol weight :
105,324 Da
ncbi pathways :
Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Glucose Metabolism Pathway (1269959); Glycogen Breakdown (glycogenolysis) Pathway (1269963); Immune System Pathway (1269170); Innate Immune System Pathway (1269203); Lysosomal Glycogen Catabolism Pathway (1269964); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956)
ncbi 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]
uniprot summary :
Essential for the degradation of glygogen to glucose in lysosomes.
size4 :
10x96-Strip-Wells