GAA Antibody / Glucosidase alpha acid

• Catalog number
  R32608
• Price
  Please ask
• Size
  0.1mg

• Category
  Antibody
• Concentration
  0.5mg/ml if reconstituted with 0.2ml sterile DI water
• Form
  Antigen affinity purified
• Conjugation
  Unconjugated
• Clone
  Polyclonal antibody
• Recognised antigen
  GAA / Glucosidase alpha acid
• Host animal
  Rabbit (Oryctolagus cuniculus)
• Clonality
  Polyclonal (rabbit origin)
• Species reactivity
  Human (Homo sapiens), Rat ; Due to limited knowledge and inability to test the antibody against all known species, we cannot guarantee that no other cross reactivity can occur.
• Tested applications
  WB, IHC-P
• Recommended dilutions
  Western blot: 0.5-1ug/ml,Immunohistochemistry (FFPE): 1-2ug/ml
• Notes
  Optimal dilution of the GAA antibody should be determined by the researcher.
• Added buffer
  Lyophilized from 1X Phosphate Buffered Saline (PBS) with 2.5% BSA and 0.025% sodium azide
• Intented use
  This GAA antibodyis to be used only for research purposes and not for diagnostics..
• Uniprot
  P10253
• Purity
  Antigen affinity
• Description
  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.
• Immunogen
  Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.
• Storage
  After reconstitution, the GAA antibody may be kept for up to one month refrigerated at +4 degrees C.For long-term, aliquot and store at -20 deg. Celcius or lower. Cycles of freezing and thawing can denaturate the peptide chains of the antibodies and reduce their sensitivity and/or change their affinity. Prepare aliqotes in such a manner so that freeze-thaw cycles are minimized. Avoid repeated freezing and thawing.
• Localization
  Cytoplasmic, membranous
• Additional description
  The Anti- GAA / Glucosidase alpha acid is a α- or alpha protein sometimes glycoprotein present in blood.
• Properties
  If you buy Antibodies supplied by NJS poly 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   Glucosidase   alpha   acid  
• 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
  Anti- GAA / Glucosidase alpha acid
• Technique
  Antibody, antibodies against human proteins, antibodies for
• Isotype
  Rabbit IgG
• Alternative name
  Antibodies to GAA / Glucosidase alpha acid
• 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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