GAA Antibody

ProSci
Product Code: PSI-23-097
Product Group: Primary Antibodies
Supplier: ProSci
CodeSizePrice
PSI-23-097-50uL50uL£433.00
Quantity:
Prices exclude any Taxes / VAT

Overview

Host Type: Rabbit
Antibody Isotype: IgG
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Shipping:
Blue Ice or RT
Storage:
Store at -20°C. Avoid freeze / thaw cycles.

Images

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Western blot analysis of extracts of various cell lines, using GAA antibody (23-097) at 1:1000 dilution.<br/>Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.<br/>Lysates/proteins: 25ug per lane.<br/>Blocking buffer: 3% nonfat dry milk in TBST.<br/>Detection: ECL Basic Kit.<br/>Exposure time: 10s.

Western blot analysis of extracts of various cell lines, using GAA antibody (23-097) at 1:1000 dilution.<br/>Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.<br/>Lysates/proteins: 25ug per lane.<br/>Blocking buffer: 3% nonfat dry milk in TBST.<br/>Detection: ECL Basic Kit.<br/>Exposure time: 10s.

Further Information

Additional Names:
Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Application Note:
WB: 1:500 - 1:2000

IF: 1:50 - 1:200

IP: 1:50 - 1:200
Background:
This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.
Buffer:
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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:
A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
NCBI Gene ID #:
2548
NCBI Official Name:
Lysosomal alpha-glucosidase
NCBI Official Symbol:
GAA
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
Observed: 105kDa
Purification:
Affinity purification
Research Area:
Cancer, Signal Transduction
Swissprot #:
P10253
User NOte:
Optimal dilutions for each application to be determined by the researcher.