Biotin-Linked Polyclonal Antibody to Glucosidase Alpha, Acid (GaA) | MyBioSource MBS2091664 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Biotin-Linked Polyclonal Antibody to Glucosidase Alpha, Acid (GaA)

catalog :

MBS2091664

quantity :

0.1 mg

price :

225 USD

clonality :

polyclonal

host :

rabbit

conjugate :

biotin

reactivity :

human

application :

western blot, immunohistochemistry, immunocytochemistry

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS2091664

products type :

Antibody

products full name :

Biotin-Linked Polyclonal Antibody to Glucosidase Alpha, Acid (GaA)

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 :

[Glucosidase, alpha; acid; Lysosomal alpha-glucosidase; lysosomal alpha-glucosidase; glucosidase alpha, acid; Acid maltase; Aglucosidase alfa]

products gene name :

[GaA]

other gene names :

[GAA; GAA; LYAG]

clonality :

Polyclonal

host :

Rabbit

reactivity :

Human

purity :

Antigen-specific Affinity Chromatography.

form :

Supplied as solution form in PBS, pH7.4, 50% glycerol 0.05% Proclin-300.

concentration :

0.87mg/ml

storage stability :

Avoid repeated freeze/thaw cycles. Store at 4 degree C for frequent use. Aliquot and store at -20 degree C for 12 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

tested application :

Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF)

app notes :

Western blotting: 0.5-2ug/mL;1:430-1700. Immunohistochemistry: 5-20ug/mL;1:43-170. Immunocytochemistry: 5-20ug/mL;1:43-170. Optimal working dilutions must be determined by end user.

other info1 :

Organism Species: Homo sapiens (Human). Source: Antibody labeling. Label: Biotin. Traits: Liquid

other info2 :

Immunogen: Recombinant GaA (Pro595~Gly770) expressed in E.coli.

ncbi gi num :

26251857

ncbi acc num :

AAH40431.1

ncbi pathways :

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolic Pathways (132956); Notch-mediated HES/HEY Network Pathway (169347); Starch And Sucrose Metabolism Pathway (82974); Starch And Sucrose Metabolism Pathway (344)

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 :

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 - galactose; Carbohydrate Metabolism - starch and sucrose; Contractile; EC 3.2.1.20; Hydrolase. Chromosomal Location of Human Ortholog: 17q25.3. Cellular Component: lysosomal lumen; lysosomal membrane; lysosome; membrane; plasma membrane. Molecular Function: alpha-1,4-glucosidase activity; carbohydrate binding; maltase activity; oligo-1,6-glucosidase activity. Biological Process: cardiac muscle contraction; diaphragm contraction; glucose metabolic process; glycogen catabolic process; heart morphogenesis; locomotory behavior; lysosome organization and biogenesis; maltose metabolic process; muscle maintenance; neuromuscular process controlling balance; neuromuscular process controlling posture; neutrophil degranulation; regulation of the force of heart contraction; sucrose metabolic process; tissue development; vacuolar sequestering. Disease: Glycogen Storage Disease Ii

size1 :

0.1 mg

price1 :

225 USD

size2 :

0.2 mg

price2 :

335

size3 :

1 mg

price3 :

795

more info or order :

MyBioSource product webpage