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Invitrogen™ GAA Recombinant Rabbit Monoclonal Antibody (5L2L10)
Rabbit Recombinant Monoclonal Antibody
Marke: Invitrogen™ MA537870
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Beschreibung
Positive Samples: 293T, HepG2, DU145 Immunogen sequence: QEQCEARGCC YIPAKQGLQG AQMGQPWCFF PPSYPSYKLE NLSSSEMGYT ATLTRTTPTF FPKDILTLRL DVMMETENRL HFTIKDPANR RYEVPLETPH V.
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.
Spezifikation
| GAA | |
| Recombinant Monoclonal | |
| 0.8 mg/mL | |
| PBS with 50% glycerol, 0.05% BSA and 0.02% sodium azide; pH 7.3 | |
| P10253 | |
| Gaa | |
| Synthetic peptide. | |
| 100 μL | |
| Primary | |
| Human | |
| Antibody | |
| IgG |
| ELISA, Western Blot | |
| 5L2L10 | |
| Unconjugated | |
| Gaa | |
| 70 kDa lysosomal alpha-glucosidase; 76 kDa lysosomal alpha-glucosidase; acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; acid maltase; Aglucosidase alfa; E430018M07Rik; Gaa; glucosidase alpha, acid; glucosidase, alpha, acid; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); LYAG; Lysosomal alpha-glucosidase | |
| Rabbit | |
| Affinity chromatography | |
| RUO | |
| 2548 | |
| -20°C, Avoid Freeze/Thaw Cycles | |
| Liquid |
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