GAA antibody (AA 350-450)
Quick Overview for GAA antibody (AA 350-450) (ABIN6140865)
Target
See all GAA AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- AA 350-450
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Sequence
- VVQQYLDVVG YPFMPPYWGL GFHLCRWGYS STAITRQVVE NMTRAHFPLD VQWNDLDYMD SRRDFTFNKD GFRDFPAMVQ ELHQGGRRYM MIVDPAISSS G
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Cross-Reactivity
- Human, Mouse, Rat
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Characteristics
- Polyclonal Antibodies
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Purification
- Affinity purification
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Immunogen
- A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
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Isotype
- IgG
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Application Notes
- WB,1:500 - 1:2000,IF,1:50 - 1:200,IP,1:50 - 1:200
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Comment
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HIGH QUALITY
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Restrictions
- For Research Use only
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Format
- Liquid
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Buffer
- PBS with 0.02 % sodium azide,50 % glycerol, pH 7.3.
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Preservative
- Sodium azide
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Precaution of Use
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Storage
- -20 °C
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Storage Comment
- Store at -20°C. Avoid freeze / thaw cycles.
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- GAA (Glucosidase, Alpha, Acid (GAA))
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Alternative Name
- GAA
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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.,GAA,LYAG,Cancer,Signal Transduction,Endocrine & Metabolism,GAA
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Molecular Weight
- 105 kDa
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Gene ID
- 2548
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UniProt
- P10253
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Pathways
- Cellular Glucan Metabolic Process
Target
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