| Mendicino,
Joseph F. |
E-mail:
mendicin@bmb.uga.edu |
| Cystic
fibrosis is a genetic disorder which results in the secretion
of very viscous mucus from cells in the trachea. This effect
ultimately leads to infection of the lungs and death. Studies
of the composition of tracheobronchial mucous glycoproteins
from patients with cystic fibrosis have shown that they exhibit
increased sulfation of longer carbohydrate chains. The metabolic
basis for the formation of these altered chains is unknown.
The primary disorder in cystic fibrosis is caused by an alteration
in membrane chloride ion transport. However increased levels
of intracellular sulfate may be a secondary consequence of electrolyte
abnormalities in this disease and may lead to increased sulfation
of longer oligosaccharide chains. Our studies are concerned
with the regulation of sulfation of these chains. |
| Keywords:
Cystic Fibrosis, sulfation, regulation, mucus, glycoproteins,
genetic defects |
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