GAA Recombinant Antibody, RBITC Conjugated
Catalogue Number: BSM-54735R-RBITC-BSS
| Manufacturer: | Bioss Inc |
| Type: | Monoclonal Primary Antibody - Conjugated |
| Shipping Condition: | Blue Ice |
| Unit(s): | 100 ul |
| Host name: | Rabbit |
| Clone: | 5H49 |
| Isotype: | IgG |
| Immunogen: | Synthetic peptide within Human GAA. |
| Application: | FC, WB |
Description
Description: 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].
Additional Text
Gene Name
GAA
Uniprot ID
P10253
Gene ID
2548
Purification
Protein A purified
Antibody Clonality
Monoclonal
Modification
Unmodified
Specificity
70 kDa lysosomal alpha-glucosidase antibody, Acid alpha glucosidase antibody, Acid maltase antibody, Aglucosidase alfa antibody, Alpha glucosidase antibody, GAA antibody, Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody, Glucosidase alpha acid antibody, Glucosidase alpha antibody, LYAG antibody, LYAG_HUMAN antibody, Lysosomal alpha glucosidase antibody
Storage Note
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Excitation
570nm/595nm
BSM-54735R-RBITC Datasheet
SUPPORT
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PRODUCT
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DELIVERY
we offer free delivery to UK universities and non profit organisations