GAA Antibody - N-terminal region (ARP44226_P050)

• Product Number: ARP44226_P050
• Name: GAA Antibody - N-terminal region (ARP44226_P050)
• Protein Size (# AA): 952 amino acids
• Molecular Weight: 98kDa
• NCBI Gene Id: 2548
• Host: Rabbit
• Clonality: Polyclonal
• Concentration: 0.5 mg/ml
• Gene Full Name: Glucosidase, alpha; acid
• Alias Symbols: LYAG
• Peptide Sequence: Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL
• Product Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Reference: Wan,L., (er) J. Neurol. (2008) In press
• Description of Target: GAA is acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.
• Protein Interactions: UBC; SUMO1; NEDD8; NUMBL; STAT2; HIVEP1; EP300; SYNCRIP; MTHFD1; ILF3; HNRNPK; CDH2; CALU; FBXO6; NCF1;
• Reconstitution and Storage: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
• Blocking Peptide: For anti-GAA (ARP44226_P050) antibody is Catalog # AAP44226 (Previous Catalog # AAPP25606)
• Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human GAA
• Uniprot ID: P10253
• Protein Name: Lysosomal alpha-glucosidase
• Sample Type Confirmation:

  GAA is supported by BioGPS gene expression data to be expressed in MCF7

• Protein Accession #: NP_000143
• Purification: Affinity Purified
• Nucleotide Accession #: NM_000152
• Tested Species Reactivity: Human
• Gene Symbol: GAA
• Predicted Species Reactivity: Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Zebrafish
• Application: IHC, WB
• Predicted Homology Based on Immunogen Sequence: Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 93%; Human: 100%; Mouse: 100%; Rabbit: 79%; Rat: 100%; Zebrafish: 83%
• Image 1: Human MCF-7
  
  WB Suggested Anti-GAA Antibody Titration: 0.2-1 ug/ml
  ELISA Titer: 1:62500
  Positive Control: MCF7 cell lysateGAA is supported by BioGPS gene expression data to be expressed in MCF7
• Image 2: Human Lung Tissue
  
  Rabbit Anti-GAA Antibody
  Catalog Number: ARP44226_P050
  Formalin Fixed Paraffin Embedded Tissue: Human Lung Tissue
  Observed Staining: Cytoplasmic in alveolar type I cells
  Primary Antibody Concentration: 1:100
  Other Working Concentrations: 1/600
  Secondary Antibody: Donkey anti-Rabbit-Cy3
  Secondary Antibody Concentration: 1:200
  Magnification: 20X
  Exposure Time: 0.5 - 2.0 sec