GAA Antibody - N-terminal region : Biotin (ARP44226_P050-Biotin)

Catalog No: ARP44226_P050-Biotin

Size:100ul

Product Info

• Predicted Species Reactivity: Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Zebrafish
• Product Format: Liquid. Purified antibody supplied in 1x PBS buffer.
• Clonality: Polyclonal
• Host: Rabbit
• Conjugation: Biotin
• Application: IHC, WB
• Reconstitution and Storage: All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil). Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.
• Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human GAA
• Purification: Affinity Purified
• 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%
• Peptide Sequence: Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL
• Concentration: 0.5 mg/ml
• Blocking Peptide: For anti-GAA (ARP44226_P050-Biotin) antibody is Catalog # AAP44226 (Previous Catalog # AAPP25606)
• Sample Type Confirmation:

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

• Reference: Wan,L., (er) J. Neurol. (2008) In press

Target Info

• Gene Symbol: GAA
• Gene Full Name: Glucosidase, alpha; acid
• Alias Symbols: LYAG
• NCBI Gene Id: 2548
• Protein Name: Lysosomal alpha-glucosidase
• 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.
• Uniprot ID: P10253
• Protein Accession #: NP_000143
• Nucleotide Accession #: NM_000152
• Protein Size (# AA): 952
• Molecular Weight: 98kDa
• Protein Interactions: UBC; SUMO1; NEDD8; NUMBL; STAT2; HIVEP1; EP300; SYNCRIP; MTHFD1; ILF3; HNRNPK; CDH2; CALU; FBXO6; NCF1;