317304
GBE1 (1,4-alpha-glucan-branching Enzyme, Brancher Enzyme, Glycogen-branching Enzyme)
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£800.00
- SKU:
- 317304
- Reinheit:
- Purified by immunoaffinity chromatography.
- Lagerbedingungen:
- -20°C
- Hersteller:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Human GBE1
- Format:
- Supplied as a liquid in PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
- Weitere Details:
- GBE1 (glucan (1,4-alpha-), branching enzyme 1) is a 702aa protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Versandbedingungen:
- Blue Ice