GAA antibody (AA 541-640) (AbBy Fluor® 647)
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- Target See all GAA Antibodies
- GAA (Glucosidase, Alpha, Acid (GAA))
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Binding Specificity
- AA 541-640
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Reactivity
- Human, Mouse, Rat
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Host
- Rabbit
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Clonality
- Polyclonal
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Conjugate
- This GAA antibody is conjugated to AbBy Fluor® 647
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Application
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- Cross-Reactivity
- Human, Mouse, Rat
- Predicted Reactivity
- Dog,Sheep,Pig,Horse
- Purification
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha
- Isotype
- IgG
- Top Product
- Discover our top product GAA Primary Antibody
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- Application Notes
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - Restrictions
- For Research Use only
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- Format
- Liquid
- Concentration
- 1 μg/μL
- Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- Preservative
- ProClin
- Precaution of Use
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- Storage
- -20 °C
- Storage Comment
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Expiry Date
- 12 months
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- Target
- GAA (Glucosidase, Alpha, Acid (GAA))
- Alternative Name
- GAA (GAA Products)
- Synonyms
- LYAG antibody, E430018M07Rik antibody, glucosidase alpha, acid antibody, glucosidase, alpha, acid antibody, transmembrane and coiled-coil domain family 1 antibody, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antibody, GAA antibody, Gaa antibody, TMCC1 antibody, gaa antibody
- Background
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Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid Pompe disease glycogen storage disease type II, Glucosidase alpha acid, Glucosidase alpha, LYAG, LYAG_HUMAN, Lysosomal alpha glucosidase.
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. [provided by RefSeq, Jul 2008].
- Gene ID
- 2548
- UniProt
- P10253
- Pathways
- Cellular Glucan Metabolic Process
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