GAA Antibody
| Code | Size | Price |
|---|
| PSI-56-495-400ul | 400ul | £626.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: Rabbit Ig
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species: Human
Applications:
- Immunohistochemistry (IHC)
- Immunohistochemistry- Paraffin Embedded (IHC-P)
- Western Blot (WB)
Storage:
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Documents
Further Information
Additional Names:
Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Application Note:
For IHC starting dilution is: 1:25
For WB starting dilution is: 1:1000
For WB starting dilution is: 1:1000
Background:
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.
Background References:
- Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
- Labrousse, P., et al. Mol. Genet. Metab. 99(4):379-383(2010)
- Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
- Aoyama, Y., et al. J. Hum. Genet. 54(11):681-686(2009)
Buffer:
Supplied in PBS with 0.09% (W/V) sodium azide.
Concentration:
batch dependent
Conjugate:
Unconjugated
DISCLAIMER:
Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Immunogen:
This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
NCBI Gene ID #:
2548
NCBI Official Name:
Lysosomal alpha-glucosidase
NCBI Official Symbol:
GAA
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
105 kDa
Protein Accession #:
P10253
Protein GI Number:
317373572
Purification:
This antibody is purified through a protein A column, followed by peptide affinity purification.
Research Area:
Obesity,Signal Transduction
Swissprot #:
P10253
User NOte:
Optimal dilutions for each application to be determined by the researcher.