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GAA Antibody (PA5-96756) in WB
Western blot analysis of GAA in extracts of various cell lines. Samples were incubated with GAA Polyclonal antibody (Product # PA5-96756) using a dilution of 1:1,000, followed by HRP Goat Anti-Rabbit IgG (H+L) at a dilution of 1:10,000. Lysates/proteins: 25 µg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 60s.
Product Details
PA5-96756
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_0001432)
Conjugate
Form
Liquid
Concentration
2.91 mg/mL
Purification
Affinity Chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.05% ProClin 300
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Wet ice
RRID
AB_2808558
Product Specific Information
Immunogen sequence: VVQQYLDVVG YPFMPPYWGL GFHLCRWGYS STAITRQVVE NMTRAHFPLD VQWNDLDYMD SRRDFTFNKD GFRDFPAMVQ ELHQGGRRYM MIVDPAISSS G; Positive Samples: LO2, HT-29, 293T, A-549, DU145, Mouse liver, Mouse heart, Mouse thymus, Rat liver; Cellular Location: Lysosome, Lysosome membrane
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; Acid maltase; Aglucosidase alfa; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); Lysosomal alpha-glucosidase
Gene Aliases: E430018M07Rik; GAA; LYAG
UniProt ID: (Human) P10253, (Mouse) P70699, (Rat) Q6P7A9
Entrez Gene ID: (Human) 2548, (Mouse) 14387, (Rat) 367562
maltose metabolic process
regulation of the force of heart contraction
diaphragm contraction
heart morphogenesis
glycogen catabolic process
sucrose metabolic process
glucose metabolic process
lysosome organization
locomotory behavior
tissue development
vacuolar sequestering
muscle cell cellular homeostasis
neuromuscular process controlling posture
neuromuscular process controlling balance
cardiac muscle contraction
carbohydrate metabolic process
glycogen metabolic process
striated muscle contraction
metabolic process
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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