Mouse anti-Human GAA Monoclonal Antibody | anti-GAA antibody

Anti-GAA Antibody (Monoclonal, 2G7)

Cat. Num. AAA19211

• Gene Names: GAA; LYAG
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry, Immunocytochemistry, Immunofluorescence, Flow Cytometry, Functional Assay
• Purity: Immunogen affinity purified.
• Synonyms: GAA; Monoclonal Antibody; Anti-GAA Antibody (Monoclonal; 2G7); Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; Glucosidase alpha acid; Glucosidase alpha; LYAG; P10253; anti-GAA antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2b
• Clone Number: 2G7
• Purity/Purification: Immunogen affinity purified.
• Form/Format: Lyophilized; Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Sequence Length: 952

• Applicable Applications for anti-GAA antibody: Western Blot (WB), Immunohistochemistry (IHC) Paraffin, Immunocytochemistry (ICC), Immunofluorescence (IF), Flow Cytometry (FC/FACS)
• Application Notes: WB: Concentration: 0.1-0.5ug/ml; Tested Species: Human; IHC-P (Embedded Section): Concentration: 0.5-1ug/ml; Tested Species: Human; ICC: Concentration: 2ug/ml; Tested Species: Human; IF: Concentration: 2ug/ml; Tested Species: Human; FC: Concentration: 1-3ug/1x10⁶ cells; Tested Species: Human; Tested Species: In-house tested species with positive results.; Other applications have not been tested.; Optimal dilutions should be determined by end users.
• Immunogen: A synthetic peptide corresponding to a sequence in the middle region of human GAA (494-527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR), different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.
• Relevant Detection Systems: QC labs recommends Enhanced Chemiluminescent Kit with anti-Mouse IgG for Western blot, and HRP Conjugated anti- Mouse IgG Super Vision Assay Kit for IHC(P) and ICC.
• Preparation and Storage: Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen store at -20 degree C for 6 months. Avoid repeated freezing and thawing.

WB (Western Blot)

IHC (Immunohistchemistry)

IHC (Immunohistochemistry)

IHC (Immunohistochemistry)

IF (Immunofluorescence)

IF (Immunofluorescence)

FCM (Flow Cytometry)

Related Product Information for anti-GAA antibody

Description: Mouse IgG monoclonal antibody for GAA detection. Tested with WB, IHC-P, ICC/IF, FCM in Human.

Background: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

References

1. "Entrez Gene: GAA glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)".
2. Donald J. Voet; Judith G. Voet; Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538.
3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61-9.

NCBI and Uniprot Product Information

• NCBI GI #: 119393891
• NCBI GeneID: 2548
• NCBI Accession #: NP_000143.2
• NCBI GenBank Nucleotide #: NP_000143.2
• UniProt Accession #: P10253
• NCBI Official Full Name: lysosomal alpha-glucosidase preproprotein
• NCBI Official Synonym Full Names: glucosidase alpha, acid
• NCBI Official Symbol: GAA
• NCBI Official Synonym Symbols: LYAG
• NCBI Protein Information: lysosomal alpha-glucosidase
• UniProt Protein Name: Lysosomal alpha-glucosidase
• UniProt Gene Name: GAA