Full Product Name
GAA Antibody, FITC conjugated
Product Synonym Names
Lysosomal alpha-glucosidase; Acid maltase; Aglucosidase alfa; 76 kDa lysosomal alpha-glucosidase; 70 kDa lyso; GAA
Product Gene Name
anti-GAA antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P10253
Purity/Purification
>95%, Protein G purified
Immunogen
Recombinant human Lysosomal alpha-glucosidase protein
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-49440 / sc-49441 / sc-67358 / sc-67359
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-GAA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-GAA antibody
Essential for the degradation of glygogen to glucose in lysosomes.
Applications Tested/Suitable for anti-GAA antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for GAA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000143.2
[Other Products]
NCBI GenBank Nucleotide #
NM_000152.4
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UniProt Primary Accession #
P10253
[Other Products]
UniProt Secondary Accession #
Q09GN4; Q14351; Q16302; Q8IWE7[Other Products]
UniProt Related Accession #
P10253[Other Products]
Molecular Weight
105,324 Da
NCBI Official Full Name
lysosomal alpha-glucosidase preproprotein
NCBI Official Synonym Full Names
glucosidase alpha, acid
NCBI Official Symbol
GAA [Similar Products]
NCBI Official Synonym Symbols
LYAG
[Similar Products]
NCBI Protein Information
lysosomal alpha-glucosidase
UniProt Protein Name
Lysosomal alpha-glucosidase
UniProt Synonym Protein Names
Acid maltase; Aglucosidase alfa
Protein Family
Lysosomal alpha-glucosidase
UniProt Gene Name
GAA [Similar Products]
UniProt Entry Name
LYAG_HUMAN
NCBI Summary for GAA
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 Comments for GAA
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 ***** forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the ***** 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: EC 3.2.1.20; Carbohydrate Metabolism - galactose; Contractile; Carbohydrate Metabolism - starch and sucrose; Hydrolase
Chromosomal Location of Human Ortholog: 17q25.2-q25.3
Cellular Component: lysosomal lumen; lysosomal membrane; lysosome; membrane
Molecular Function: alpha-glucosidase activity; oligo-1,6-glucosidase activity
Biological Process: cardiac muscle contraction; diaphragm contraction; glucose metabolic process; glycogen catabolic process; lysosome organization and biogenesis; maltose metabolic process; sucrose metabolic process; vacuolar sequestering
Disease: Glycogen Storage Disease Ii
Research Articles on GAA
1. G GAA gene mutation in homozygous state. All patients had decreased GAA activity and elevated creatine kinase levels.">Study reports on the clinical, biochemical, morphological, muscle imaging, and genetic findings of six ***** Pompe patients from five unrelated families with the c.-32-13T>G GAA gene mutation in homozygous state. All patients had decreased GAA activity and elevated creatine kinase levels.
Precautions
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