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WB (Western Blot)
Mouse anti-Human GAA Monoclonal Antibody | anti-GAA antibody
Anti-GAA Antibody (Monoclonal, 2G7)
Gene Names
GAA; LYAG
Reactivity
Human
Applications
Western Blot, Immunohistochemistry, Immunocytochemistry, Immunofluorescence, Flow Cytometry, Functional Assay
Purity
Immunogen affinity purified.
Synonyms
GAA, Antibody; Anti-GAA Antibody (Monoclonal, 2G7); Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; 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.
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.
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)
WB (Western Blot)
IHC (Immunohistchemistry)
IHC (Immunohistchemistry)
IHC (Immunohistochemistry)
IHC (Immunohistochemistry)
IHC (Immunohistochemistry)
IHC (Immunohistochemistry)
IF (Immunofluorescence)
IF (Immunofluorescence)
IF (Immunofluorescence)
IF (Immunofluorescence)
FCM (Flow Cytometry)
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.
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.
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 #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
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
Similar Products
Product Notes
The GAA gaa (Catalog #AAA19211) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Anti-GAA Antibody (Monoclonal, 2G7) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's GAA can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC) Paraffin, Immunocytochemistry (ICC), Immunofluorescence (IF), Flow Cytometry (FC/FACS). 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. Researchers should empirically determine the suitability of the GAA gaa for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GAA, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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