Human Lysosomal alpha-glucosidase, GAA ELISA Kit | MyBioSource MBS947492 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Lysosomal alpha-glucosidase, GAA ELISA Kit

catalog :

MBS947492

quantity :

48-Strip-Wells

price :

510 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS947492

products type :

ELISA Kit

products full name :

Human Lysosomal alpha-glucosidase, GAA ELISA Kit

products short name :

glucosidase, alpha; acid

products name syn :

Human Lysosomal alpha-glucosidase (GAA) ELISA kit; LYAG; acid alpha-glucosidase; acid maltase; lysosomal alpha-glucosidase; glucosidase; alpha; acid

other names :

lysosomal alpha-glucosidase preproprotein; Lysosomal alpha-glucosidase; lysosomal alpha-glucosidase; acid maltase; aglucosidase alfa; glucosidase, alpha; acid; Acid maltase; Aglucosidase alfaCleaved into the following 2 chains:76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase

products gene name :

GAA

other gene names :

GAA; GAA; LYAG

uniprot entry name :

LYAG_HUMAN

reactivity :

Human

sequence length :

952

specificity :

This assay has high sensitivity and excellent specificity for detection of human GAA. No significant cross-reactivity or interference between human GAA and analogues was observed.

storage stability :

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

other info1 :

Samples: Serum, plasma, tissue homogenates, cell lysates. Assay Type: Sandwich. Detection Range: 25 pg/ml -1600 pg/ml. Sensitivity: The minimum detectable dose of human GAA is typically less than 6.25 pg/ml. The sensitivity of this assay, or Lower Limit of Detection (LLD) was defined as the lowest protein concentration that could be differentiated from zero. It was determined the mean O.D value of 20 replicates of the zero standard added by their three standard deviations.

other info2 :

Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.

products description :

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for GAA has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any GAA present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for GAA is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of GAA bound in the initial step. The color development is stopped and the intensity of the color is measured.

ncbi gi num :

119393891

ncbi acc num :

NP_000143.2

ncbi gb acc num :

NM_000152.3

uniprot acc num :

P10253

ncbi mol weight :

105,324 Da

ncbi pathways :

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Lysosome Pathway (99052); Lysosome Pathway (96865); Notch-mediated HES/HEY Network Pathway (169347); Starch And Sucrose Metabolism Pathway (82974); Starch And Sucrose Metabolism Pathway (344)

ncbi summary :

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. [provided by RefSeq, Jul 2008]

uniprot summary :

GAA: Essential for the degradation of glygogen to glucose in lysosomes. Defects in GAA are the cause of glycogen storage disease type 2 (GSD2); also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. Belongs to the glycosyl hydrolase 31 family. Protein type: Carbohydrate Metabolism - starch and sucrose; EC 3.2.1.20; Hydrolase; Carbohydrate Metabolism - galactose; Contractile. Chromosomal Location of Human Ortholog: 17q25.2-q25.3. Cellular Component: membrane; lysosomal membrane; lysosome. Molecular Function: alpha-glucosidase activity; maltase activity; carbohydrate binding. Biological Process: heart morphogenesis; tissue development; maltose metabolic process; glycogen catabolic process; vacuolar sequestering; glucose metabolic process; locomotory behavior; sucrose metabolic process; muscle maintenance; neuromuscular process controlling posture; lysosome organization and biogenesis; diaphragm contraction; neuromuscular process controlling balance; regulation of the force of heart contraction; cardiac muscle contraction. Disease: Glycogen Storage Disease Ii

size1 :

48-Strip-Wells

price1 :

510 USD

size2 :

96-Strip-Wells

price2 :

725

size3 :

5x96-Strip-Wells

price3 :

2565

size4 :

10x96-Strip-Wells

price4 :

4800

more info or order :

MyBioSource product webpage