Cystic fibrosis and the salt controversy

Article Abstract:

Cystic fibrosis (CF) is a disease that is characterized by chronic bacterial infection of the lungs. With symptoms that include impaired mucociliary clearance, chronic infections and lung inflammation, CF is believed to arise when the human airway's defense systems break down, leading to infection. Two seemingly contradictory models of CF infection are analyzed. One model, known as the hydration model suggests that more lung liquid is needed to treat respiratory problems associated with CF, while the other, the salt hypothesis, suggests that salt imbalances in the airway need to corrected.

author: Guggino, William B.
Care and treatment, Physiological aspects, Bacterial infections, Lung diseases, Cystic fibrosis, Respiratory tract diseases, Mucus

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CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

Article Abstract:

The cystic fibrosis transmembrane conductance regulator (CFTR) stimulated by cyclic AMP and protein kinase A (PKA) regulates the conductance of chloride ions and either releases ATP out of the cell or regulates an ATP release channel. Secretory epithelia from patients with cystic fibrosis (CF) exhibit low chloride ion conductance. CFTR also regulates other transport pathways as patients with CF have increased sodium ion absorption which is related to a wild-type CFTR. However, the cellular mechanism of CFTR action is uncertain.

author: Cutting, Garry R., Guggino, William B., Schwiebert, Erik M., Egan, Marie E., Hwang, Tae-Ho, Fulmer, Stephanie B., Allen, Sandra S.
Observations, Enzymes, Cyclic adenylic acid, Cyclic adenosine monophosphate, Enzyme regulation, Chloride channels

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Coassembly of TRP and TRPL produces a distinct store-operated conductance

Article Abstract:

In vivo and in vitro assays of transient receptor potential (TRP) and TRPL were analyzed to determine the interaction between these two proteins during coimmunoprecipitation in 293T cells. Results indicate that the coassembly of TRP and TRPL induces a distinct store-operated conductance. Furthermore, the two proteins' direct interaction suggest that the TRP-TRPL-dependent current is connected by the heteromultimeric relationship between the two subunits.

author: Guggino, William B., Montell, Craig, Xu, Xian-Zhong Shawn, Li, Hong Sheng
Proteins, Photoreceptors

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subjects list: Research
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