GAA Antibody / Glucosidase alpha acid

NSJ Bioreagents
Product Code: NSJ-F54279
Product Group: Primary Antibodies
Supplier: NSJ Bioreagents
CodeSizePrice
NSJ-F54279-0.08ML0.08 ml£260.00
Quantity:
NSJ-F54279-0.4ML0.4 ml£502.00
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- Paraffin Embedded (IHC-P)
  • Western Blot (WB)
Storage:
Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Images

1 / 5
Western blot testing of human 1) HeLa, 2) MCF7, 3) SW620 and 4) A549 cell lysate with GAA antibody. Predicted molecular weight ~105 kDa.
2 / 5
Western blot testing of human 1) A549, 2) MCF7, 3) SW620 and 4) PC-3 cell lysate with GAA antibody. Predicted molecular weight ~105 kDa.
3 / 5
Western blot testing of human placenta lysate with GAA antibody. Predicted molecular weight ~105 kDa.
4 / 5
IHC testing of FFPE human placenta tissue with GAA antibody. HIER: steam section in pH6 citrate buffer for 20 min and allow to cool prior to staining.
5 / 5
IHC testing of FFPE human liver tissue with GAA antibody. HIER: steam section in pH6 citrate buffer for 20 min and allow to cool prior to staining.

Western blot testing of human 1) HeLa, 2) MCF7, 3) SW620 and 4) A549 cell lysate with GAA antibody. Predicted molecular weight ~105 kDa.
Western blot testing of human 1) A549, 2) MCF7, 3) SW620 and 4) PC-3 cell lysate with GAA antibody. Predicted molecular weight ~105 kDa.
Western blot testing of human placenta lysate with GAA antibody. Predicted molecular weight ~105 kDa.
IHC testing of FFPE human placenta tissue with GAA antibody. HIER: steam section in pH6 citrate buffer for 20 min and allow to cool prior to staining.
IHC testing of FFPE human liver tissue with GAA antibody. HIER: steam section in pH6 citrate buffer for 20 min and allow to cool prior to staining.

Further Information

Application Details :
Western blot: 1:500-1:2000,Immunohistochemistry (FFPE): 1:25
Application Note:
The stated application concentrations are suggested starting points. Titration of the GAA antibody may be required due to differences in protocols and secondary/substrate sensitivity.
Description:
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.
Format :
Purified
Formulation :
In 1X PBS, pH 7.4, with 0.09% sodium azide
Immunogen:
A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.
Limitation:
This GAA antibody is available for research use only.
Purity:
Antigen affinity purified
Species Reactivity :
Human
Uniprot #:
P10253