Lysosomal α-Glucosidase (GAA) Activity Assay Kit (Fluorometric)

• Catalog number
  K187-100
• Price
  Please ask
• Size
  100 assays

• Description
  Highly Sensitive α-Glucosidase Activity Assay
• Summary
  • Detection method- Fluorescence (368/460 nm) • Applications: - Measurement of lysosomal α-Glucosidase activity in biological samples - Assay uses a specific substrate for the detection of GAA activity
• Detection Method
  Fluorescence (368/460 nm)
• Species Reactivity
  Eukaryotes
• Applications
  · Measurement of Lysosomal α-Glucosidase activity in various tissues/cells.
• Sample Type
  • Tissue Homogenates: Muscle, Heart, etc. • Cell Lysates: 3T3 Cell Lysates, etc. • Biological fluids: Serum, etc.
• Features Benefits
  • Rapid and easy-to-use. • Highly-sensitive & high-throughput capable • This assay kit can measure0.05 µU of α-glucosidase activity in a variety of sample.
• Storage Conditions
  -20°C
• Shipping Conditions
  gel pack
• Shelf life
  12 months
• Background
  Lysosomal α-Glucosidase (GAA, Acid α-Glucosidase, Acid Maltase; EC 3.2.1.3) is an exo-1,4 and exo-1,6-α-Glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Deficiency in GAA activity results in the accumulation of glycogen within the lysosome, leading to Glycogen storage disease type II [also termed acid maltase deficiency (AMD) or Pompe disease]. Pompe disease is an inherited disorder of glycogen metabolism with broad spectrum of clinical phenotypes. Cardiac and skeletal muscles are the major target tissues and GAA activity in dry blood spots has been used to screen, and diagnose Pompe Disease. BioVision’s Lysosomal α-Glucosidase (GAA) Activity Assay Kit provides a simple way to monitor GAA activity in a wide variety of Biological Samples. In this kit, GAA cleaves a synthetic specific substrate releasing a fluorophore, which can be easily quantified (Ex/Em= 368/460 nm). The assay is specific, sensitive- it can detect as low as 0.05 µU of GAA activity
• Data Sheet Link
• Test
  Biovision supplies other types of Assays as 1.

• Gene target
  Lysosomal   α-Glucosidase   GAA   Activity   Kit   Fluorometric  
• 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
  Lysosomal α-Glucosidase (GAA) Activity Assay Kit (Fluorometric)
• Technique
  Assay, assays
• Host
  Assay
• Alternative name
  Lysosomal α-Glucosidase (GAA) Activity test reagent (fluorescence)
• Alternative technique
  arrays, kits
• Alternative to gene target
  v-kit Hardy-Zuckerman 4 feline sarcoma viral oncogene homolog, C-Kit and CD117 and PBT and SCFR, KIT and IDBG-18980 and ENSG00000157404 and 3815, transferase activity, Extracellular, Kit and IDBG-172083 and ENSMUSG00000005672 and 16590, KIT and IDBG-642326 and ENSBTAG00000002699 and 280832

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

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