- Gene Symbol:
- GAA
- Official Gene Full Name:
- Glucosidase, alpha; acid
- NCBI Gene Id:
- 2548
- Protein Name:
- Lysosomal alpha-glucosidase
- Swissprot Id:
- P10253
- Protein Accession #:
- NP_000143
- Nucleotide Accession #:
- NM_000152
- Alias Symbols:
- LYAG
- Replacement Item:
- This antibody may replace item sc-115825 from Santa Cruz Biotechnology.
- 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 Size (# AA):
- 952
- Molecular Weight:
- 98kDa
- Host:
- Rabbit
- Clonality:
- Polyclonal
- Purification:
- Affinity Purified
- Application:
- IHC, WB
- Tissue Tool:
- Find tissues and cell lines supported by DNA array analysis to express GAA.
- RNA Seq:
- Find tissues and cell lines supported by RNA-seq analysis to express GAA.
- Immunogen:
- The immunogen is a synthetic peptide directed towards the N terminal region of human GAA
- Predicted Species Reactivity:
- Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish
- Tested Species Reactivity:
- Human
- 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%
- Complete computational species homology data:
- Anti-GAA (ARP44226_P050)
- 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.
- 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.
- Concentration:
- Batch dependent within range: 100 ul at 0.5 - 1 mg/ml
- Protein Interactions:
- UBC; SUMO1; NEDD8; NUMBL; STAT2; HIVEP1; EP300; SYNCRIP; MTHFD1; ILF3; HNRNPK; CDH2; CALU; FBXO6; NCF1;
- Blocking Peptide:
- For anti-GAA (ARP44226_P050) antibody is Catalog # AAP44226 (Previous Catalog # AAPP25606)
- Datasheets/Manuals:
- Printable datasheet for anti-GAA (ARP44226_P050) antibody
- Sample Type Confirmation:
GAA is supported by BioGPS gene expression data to be expressed in MCF7
- Target Reference:
- Wan,L., (er) J. Neurol. (2008) In press
Product Reviews
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