GAA Antibody

• Catalog number
  A01548
• Price
  Please ask
• Size
  0,1 mg

• Target antigen
  GAA
• Clonality
  Polyclonal antibody
• Clone
  Polyclonal antibody
• Raised in
  rabbit
• Type of the antibody
  IgG polyclonal antibody
• Product form
  freeze-dried
• Reacts with species
  mouse, rat
• Analyses
  WB,IHC-P
• 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.
• Product configuration
  Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Purification
  Immunogen affinity purified.
• Solubilization
  The powdered antibody should be dissolved in 0.2 ml of distilled water to achieve final concentration of 500ug/ml
• Storage condtions
  Keep the GAA Antibodyat minus twenty degrees Celsius for 1 year. The ready-to-use solutions can be stored at four degrees Celsius for a month. Our specialsits recommend to freeze the aliquotes at minus twenty degrees Celsius for long-term application. Multiple procedures of freezing and thawing influence the specifity and reactivity of the antibody in a negative way.
• Tips
  The GAA Antibody did not cross-reacted with other proteins during the test procedures. This antobdy is intended to be used for research analyses and it is not applicale for in vitro diagnostics.
• 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.
• Related articles
  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.
• Gene Name
  GAA
• Protein Name
  Lysosomal alpha-glucosidase
• Gene Full Name
  glucosidase alpha, acid
• Synonyms
  Acid alpha glucosidase | Acid maltase | Aglucosidase alfa | Alpha glucosidase | GAA | Glucosidase alpha acid | Glucosidase alpha | LYAG | P10253
• Uniprot ID
  P10253
• Entrez GeneID
  2548
• Properties
  If you buy Antibodies supplied by boster they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
• French translation
  anticorps

• Gene target
  GAA  
• Gene symbol
  GAA, TRF-GAA12-1, TRF-GAA4-1, TRF-GAA8-1, TRF-GAA7-1, TRF-GAA10-1, TRF-GAA3-1, TRF-GAA6-1, TRF-GAA9-1, TRF-GAA2-1
• Short name
  GAA Antibody
• Technique
  Antibody, antibodies against human proteins, antibodies for
• Alternative name
  GAA (antibody to-)
• Alternative technique
  antibodies

Gene info

• Identity
  HGNC:4065
• Gene
  GAA
• Long gene name
  alpha glucosidase
• Synonyms gene name
  • glucosidase, alpha; acid
  • glucosidase alpha, acid
• Synonyms name
  • Pompe disease
  • glycogen storage disease type II
• Locus
  17q25.3
• Discovery year
  1986-01-01
• Entrez gene record
  2548
• Classification
  • Glycoside hydrolase family 31
• VEGA ID
  OTTHUMG00000177537
• Locus Specific Databases
  • GAA - Pompe disease (glycogen storage disease type II)
  • Global Variome shared LOVD
  • LRG_673

Gene info

• Identity
  HGNC:35044
• Gene
  TRF-GAA12-1
• Long gene name
  tRNA-Phe (anticodon GAA) 12-1
• Synonyms gene
  • TRNAF14P
• Synonyms gene name
  • transfer RNA phenylalanine 14 (anticodon GAA) pseudogene
  • transfer RNA-Phe (GAA) 12-1
• Synonyms
  • tRNA-Phe-GAA-12-1
• GenBank acession
  • HG983976
• Locus
  8q24.13
• Discovery year
  2008-08-29
• Entrez gene record
  100189471
• RefSeq identity
  • NG_008529
• Classification
  • Cytoplasmic transfer RNA pseudogenes

Gene info

• Identity
  HGNC:34859
• Gene
  TRF-GAA4-1
• Long gene name
  tRNA-Phe (anticodon GAA) 4-1
• Synonyms gene
  • TRNAF9
• Synonyms gene name
  • transfer RNA phenylalanine 9 (anticodon GAA)
  • transfer RNA-Phe (GAA) 4-1
• Synonyms
  • tRNA-Phe-GAA-4-1
• GenBank acession
  • HG983968
• Locus
  6p22.1
• Discovery year
  2008-08-29
• Entrez gene record
  100189293
• Classification
  • Cytoplasmic transfer RNAs

Gene info

• Identity
  HGNC:35016
• Gene
  TRF-GAA8-1
• Long gene name
  tRNA-Phe (anticodon GAA) 8-1
• Synonyms gene
  • TRNAF13P
• Synonyms gene name
  • transfer RNA phenylalanine 13 (anticodon GAA) pseudogene
  • transfer RNA-Phe (GAA) 8-1
• Synonyms
  • tRNA-Phe-GAA-8-1
• GenBank acession
  • HG983972
• Locus
  6q14.1
• Discovery year
  2008-08-29
• Entrez gene record
  100189446
• Classification
  • Cytoplasmic transfer RNA pseudogenes

Gene info

• Identity
  HGNC:34990
• Gene
  TRF-GAA7-1
• Long gene name
  tRNA-Phe (anticodon GAA) 7-1
• Synonyms gene
  • TRNAF12
• Synonyms gene name
  • transfer RNA phenylalanine 12 (anticodon GAA)
• Synonyms
  • tRNA-Phe-GAA-7-1
• GenBank acession
  • HG983971
• Locus
  6p22.1
• Discovery year
  2008-08-29
• Entrez gene record
  100189421
• Classification
  • Cytoplasmic transfer RNA pseudogenes

Gene info

• Identity
  HGNC:35072
• Gene
  TRF-GAA10-1
• Long gene name
  tRNA-Phe (anticodon GAA) 10-1
• Synonyms gene
  • TRNAF16P
• Synonyms gene name
  • transfer RNA phenylalanine 16 (anticodon GAA) pseudogene
  • transfer RNA-Phe (GAA) 10-1
  • tRNA-Phe (GAA) 10-1
• Synonyms
  • tRNA-Phe-GAA-10-1
• GenBank acession
  • HG983974
• Locus
  1q21.2
• Discovery year
  2008-08-29
• Entrez gene record
  100189497
• RefSeq identity
  • NG_008555
• Classification
  • Cytoplasmic transfer RNA pseudogenes

Gene info

• Identity
  HGNC:34732
• Gene
  TRF-GAA3-1
• Long gene name
  tRNA-Phe (anticodon GAA) 3-1
• Synonyms gene
  • TRNAF5
• Synonyms gene name
  • transfer RNA phenylalanine 5 (anticodon GAA)
  • transfer RNA-Phe (GAA) 3-1
• Synonyms
  • tRNA-Phe-GAA-3-1
• GenBank acession
  • HG983967
• Locus
  6p22.1
• Discovery year
  2008-08-29
• Entrez gene record
  100189170
• Classification
  • Cytoplasmic transfer RNAs

Gene info

• Identity
  HGNC:34825
• Gene
  TRF-GAA6-1
• Long gene name
  tRNA-Phe (anticodon GAA) 6-1
• Synonyms gene
  • TRNAF8
• Synonyms gene name
  • transfer RNA phenylalanine 8 (anticodon GAA)
  • transfer RNA-Phe (GAA) 6-1
• Synonyms
  • tRNA-Phe-GAA-6-1
• GenBank acession
  • HG983970
• Locus
  6p22.1
• Discovery year
  2008-08-29
• Entrez gene record
  100189262
• Classification
  • Cytoplasmic transfer RNAs

Gene info

• Identity
  HGNC:35110
• Gene
  TRF-GAA9-1
• Long gene name
  tRNA-Phe (anticodon GAA) 9-1
• Synonyms gene
  • TRNAF17P
• Synonyms gene name
  • transfer RNA phenylalanine 17 (anticodon GAA) pseudogene
  • transfer RNA-Phe (GAA) 9-1
• Synonyms
  • tRNA-Phe-GAA-9-1
• GenBank acession
  • HG983973
• Locus
  1q21.2
• Discovery year
  2008-08-29
• Entrez gene record
  100189533
• RefSeq identity
  • NG_008591
• Classification
  • Cytoplasmic transfer RNA pseudogenes

Gene info

• Identity
  HGNC:33255
• Gene
  TRF-GAA2-1
• Long gene name
  tRNA-Phe (anticodon GAA) 2-1
• Synonyms gene
  • TRNAF1
• Synonyms gene name
  • transfer RNA phenylalanine 1 (anticodon GAA)
  • transfer RNA-Phe (GAA) 2-1
  • tRNA-Phe (GAA) 2-1
• Synonyms
  • tRNA-Phe-GAA-2-1
• GenBank acession
  • HG983966
• Locus
  11q12.1
• Discovery year
  2007-02-01
• Entrez gene record
  100009605
• Pubmed identfication
  • 3678837
• Classification
  • Cytoplasmic transfer RNAs

MeSH Data

• Name
  Blotting, Western
• Concept
  Scope note: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
• Tree numbers
  • E05.196.401.143
  • E05.301.300.096
  • E05.478.566.320.200
  • E05.601.262
  • E05.601.470.320.200
• Qualifiers
  ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data

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