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LYAG rabbit pAb

CAT: 0855-ES15073-01Size: 50 µLDry Ice: NoHazardous: No
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CAT#:0855-ES15073-01Size:50 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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. [provided by RefSeq, Jan 2016]
Description
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. [provided by RefSeq, Jan 2016],
Product Name Alternative
Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase]
UniProt
P10253
Swiss Prot
P10253
Reactivity
Human; Mouse; Rat
Immunogen
Synthesized peptide derived from human LYAG AA range: 432-482
Target
LYAG
Clonality
Polyclonal
Source
Rabbit
Applications
WB
Concentration
1 mg/ml
Dilution
WB 1:500-2000
Buffer
-20°C/1 year
Molecular Weight
105kD
Storage Conditions
-20°C/1 year
Observed Molecular Weight
105kD
Fragment
IgG
Subcellular Location
Lysosome . Lysosome membrane .
Other Product Names
Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase]
Gene ID (Human)
2548

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