AGLU antibody (N-Term)
Quick Overview for AGLU antibody (N-Term) (ABIN950380)
Target
See all AGLU AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- AA 173-203, N-Term
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Specificity
- This antibody recognizes Human Alpha-glucosidase (N-term).
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Purification
- Protein A column, followed by peptide affinity purification
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Immunogen
- conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of Human Alpha-glucosidase Genename: GAA
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Isotype
- Ig Fraction
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Application Notes
- Optimal working dilution should be determined by the investigator.
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Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 0.25 mg/mL
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Buffer
- PBS containing 0.09 % (W/V) Sodium Azide as preservative
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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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Handling Advice
- Avoid repeated freezing and thawing.
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Storage
- 4 °C/-20 °C
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Storage Comment
- Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
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- AGLU (alpha-Glucosidase (AGLU))
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Alternative Name
- alpha-Glucosidase
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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.Synonyms: Acid maltase, Aglucosidase alfa, GAA, LYAG, Lysosomal alpha-glucosidase
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Molecular Weight
- 105319 Da
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Gene ID
- 2548
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NCBI Accession
- NP_000143
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Pathways
- Cellular Glucan Metabolic Process
Target
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