Alpha-glucosidase / LYAG antibody

Principal name

Alpha-glucosidase / LYAG antibody

Alternative names for Alpha-glucosidase / LYAG antibody

Acid maltase, Aglucosidase alfa, GAA, Lysosomal alpha-glucosidase

SwissProt ID

P10253 (Human), P70699 (Mouse), Q6P7A9 (Rat), Q9MYM4 (Bovin)

Gene ID

2548 (GAA)

Available reactivities

Hu (Human), Ms (Mouse), Bov (Bovine), Can (Canine), Rt (Rat), GP (Guinea Pig), Por (Porcine)

Available hosts

Mouse, Rabbit

Available applications

Enzyme Immunoassay (E), Western blot / Immunoblot (WB), ELISA (detection) (E(detection)), Immunocytochemistry/Immunofluorescence (ICC/IF), Paraffin Sections (P)

Background of Alpha-glucosidase / LYAG antibody

Lysosomal α -glucosidase, like all other lysosomal enzymes of which the 'life-cycle' has been studied, is synthesized as a large precursor that is processed to mature forms of lower molecular mass.
Acid α -glucosidase catalyzes the hydrolysis of the α1 -> 4 and α1 -> 6 glucosidic linkages in glycogen and the α1 -> 4 glucosidic linkage in maltose and artificial substrates likep-nitrophenyl- α -glucoside. The enzyme is deficient in patients with Glycogenosis Type II (Pompe's disease).
Pompe disease (also called Glycogen storage disease type II (GSD II) or acid maltase deficiency) is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism.
The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and nervous system.
There are exceptions but levels of alpha-glucosidase determines the type of GSD II an individual may have. More alpha glucosidase present in the individuals muscles means symptoms occur later in life and progress more slowly. GSD II is broadly divided into two onset forms based on the age symptoms occur: 
• Infantile-onset form is usually diagnosed at 4-8 months; muscles appear normal but are limp and weak preventing them from lifting their head or rolling over. As the disease progresses heart muscles thicken and progressively fail. Without treatment death usually occurs due to heart failure and respiratory weakness. 
• Late/later onset form occurs later than one to two years and progresses more slowly than Infantile-onset form. One of the first symptoms is a progressive decrease in muscle strength starting with the legs and moving to smaller muscles in the trunk and arms, such as the diaphragm and other muscles required for breathing. Respiratory failure is the most common cause of death. Enlargement of the heart muscles and rhythm disturbances are not significant features but do occur in some cases.

The disease is caused by a mutation in a gene (acid alpha-glucosidase: also known as acid maltase) on long arm of chromosome 17 at 17q25.2-q25.3 (base pair 75,689,876 to 75,708,272). The number of mutations described is currently (in 2010) 289 with 67 being non-pathogenic mutations and 197 pathogenic mutations. The remainder are still being evaluated for their association with disease.

General readings

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Labrousse, P., et al. Mol. Genet. Metab. 99(4):379-383(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Aoyama, Y., et al. J. Hum. Genet. 54(11):681-686(2009)
Maimaiti, M., et al. J. Hum. Genet. 54(8):493-496(2009)

20 Item(s)

per page

Primary Antibodies

Catalog No. Host Iso. Clone Pres. React. Applications  

Alpha-glucosidase / LYAG antibody

WB Suggested Anti-GAA Antibody Titration: 0.2-1 ug/ml; ELISA Titer: 1: 62500; Positive Control: MCF7 cell lysate;GAA is supported by BioGPS gene expression data to be expressed in MCF7 Rabbit IgG Purified Bov, Can, GP, Hu, Ms, Por, Rt WB
50 µg / €325.00
  OriGene Technologies, Inc.

Alpha-glucosidase / LYAG antibody

Formalin-Fixed, Paraffin-Embedded Human Placenta tissue stained with GAA Antibody Cat.-No AM32801 Mouse IgG1 43G7 Purified Hu P, WB
0.1 mg / €320.00
  Acris Antibodies GmbH

Alpha-glucosidase / LYAG (N-term) antibody

Immunohistochemistry analysis in formalin fixed and paraffin embedded human lung carcinoma reacted with Alpha-glucosidase Antibody (N-term) Cat.-No AP51752PU-N followed by peroxidase conjugation of the secondary antibody and DAB staining. Rabbit Ig Aff - Purified Hu P, WB
0.4 ml / €370.00
  Acris Antibodies GmbH

Alpha-glucosidase / LYAG antibody

Western Blot analysis of GAA expression in transfected 293T cell line (H00002548-T01) by GAA MaxPab polyclonal antibody.

Lane 1: GAA transfected lysate(104.72 KDa).
Lane 2: Non-transfected lysate. Mouse Purified Hu E(detection), WB
50 µg / €370.00
  Abnova Taiwan Corp.

Alpha-glucosidase / LYAG antibody

GAA MaxPab rabbit polyclonal antibody. Western Blot analysis of GAA expression in mouse intestine. Rabbit Purified Hu, Ms E(detection), WB
0.1 mg / €380.00
  Abnova Taiwan Corp.

Alpha-glucosidase / LYAG antibody

Alpha-glucosidase / LYAG Mouse IgG1 3C6 Purified Hu E, WB
0.1 mg / €370.00
  Abnova Taiwan Corp.

Alpha-glucosidase / LYAG antibody

Human Lung Tissue; Rabbit Anti-GAA Antibody . Catalog Number: ARP44226_P050 . Formalin Fixed Paraffin Embedded Tissue: Human Lung Tissue . Observed Staining: Cytoplasmic in alveolar type I cells . Primary Antibody Concentration: 1:100 . Other Working Concentrations: 1/600 . Secondary Antibody: Donkey anti-Rabbit-Cy3 . Secondary Antibody Concentration: 1:200 . Magnification: 20X . Exposure Time: 0.5 - 2.0 sec .; Rabbit Anti-GAA Antibody (ARP44226_P050) ) in Human Lung Tissue using Immunohistochemistry Rabbit Aff - Purified Bov, Can, Hu, Ms, Rt WB
50 µg / €350.00
  AVIVA Systems Biology

Alpha-glucosidase / LYAG antibody

Western Blot: GAA Antibody [NBP1-69295] - This Anti-GAA antibody was used in Western Blot of MCF7 tissue lysate at a concentration of 1ug/ml. Rabbit Aff - Purified Bov, Can, Hu, Ms, Rt WB
50 µg / €440.00
  Novus Biologicals Inc.

Proteins & Growth Factors

Catalog No. Species Pres. Purity Source  

Alpha-glucosidase / LYAG (transcript variant 3)

Alpha-glucosidase / LYAG Human > 80 %
Preparation: Recombint protein was captured through anti-DDK affinity column followed by conventiol chromatography steps.
Purity Detail: > 80% as determined by SDS-PAGE and Coomassie blue staining.
HEK293 cells
20 µg / €680.00
  OriGene Technologies, Inc.

Alpha-glucosidase / LYAG (transcript variant 1)

Alpha-glucosidase / LYAG Human > 80 %
Preparation: Recombint protein was captured through anti-DDK affinity column followed by conventiol chromatography steps.
Purity Detail: > 80% as determined by SDS-PAGE and Coomassie blue staining.
HEK293 cells
20 µg / €680.00
  OriGene Technologies, Inc.

Alpha-glucosidase / LYAG (transcript variant 2)

Alpha-glucosidase / LYAG Human > 80 %
Preparation: Recombint protein was captured through anti-DDK affinity column followed by conventiol chromatography steps.
Purity Detail: > 80% as determined by SDS-PAGE and Coomassie blue staining.
HEK293 cells
20 µg / €680.00
  OriGene Technologies, Inc.

Alpha-glucosidase / LYAG

Alpha-glucosidase / LYAG Human Purified 12.5% SDS-PAGE Stained with Coomassie Blue.
10 µg / €380.00
  Abnova Taiwan Corp.

Alpha-glucosidase / LYAG

Alpha-glucosidase / LYAG Human Purified 12.5% SDS-PAGE Stained with Coomassie Blue.
25 µg / €510.00
  Abnova Taiwan Corp.

Alpha-glucosidase / LYAG

Alpha-glucosidase / LYAG Human 12.5% SDS-PAGE Stained with Coomassie Blue. in vitro transl.
10 µg / €380.00
  Abnova Taiwan Corp.

Alpha-glucosidase / LYAG

Alpha-glucosidase / LYAG Human 12.5% SDS-PAGE Stained with Coomassie Blue. in vitro transl.
25 µg / €510.00
  Abnova Taiwan Corp.

Lysates

Catalog No.    

GAA 293T Cell Transient Overexpression Lysate(Denatured)

GAA 293T Cell Transient Overexpression Lysate(Denatured)  
0.1 ml / €270.00
  Abnova Taiwan Corp.

GAA overexpression lysate

GAA overexpression lysate  
0.1 mg / €280.00
  OriGene Technologies, Inc.

GAA overexpression lysate

GAA overexpression lysate  
0.1 mg / €480.00
  OriGene Technologies, Inc.

GAA overexpression lysate

GAA overexpression lysate  
0.1 mg / €280.00
  OriGene Technologies, Inc.

GAA overexpression lysate

GAA overexpression lysate  
0.1 mg / €480.00
  OriGene Technologies, Inc.

20 Item(s)

per page
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