Cystic fibrosis: a new theory

[Guest guest]

Hi,

if those scientists only could come up with a solution instead of developing

new theories all the time :-(

But then I remind myself of the motto of another CF-dad that sounded like

" Every night while you are asleep someone on this earth is working on a cure

for CF "

Peace

Torsten, dad of Fiona 6wcf

e-mail: torstenkrafft@...

> Cystic fibrosis: a new theory

> Scientists have proposed a new theory for the infections that are

> found in cases of cystic fibrosis [5]. The researchers, at the

> University of Michigan Medical School (Ann Arbor, MI, USA) and the

> University of New Mexico Health Sciences Centre (Albuquerque, NM,

> USA), showed that the gene which is mutated in cystic fibrosis (CF)

> can alter the internal chemistry of lung cells, making them more

> susceptible to certain pathogenic bacteria.

>

> `This research proposes a novel explanation for why lung infections

> are so persistent in CF,' said at the National

> Institute of Allergy and Infectious Diseases (NIAID), which funded the

> study.

>

> Cystic fibrosis causes chronic respiratory infections and sufferers

> ultimately die from a build-up of Pseudomonas aeruginosa, which rarely

> causes disease in healthy humans. The CF gene, known since 1989,

> encodes for the CF transmembrane conductance regulator (CFTR), which

> regulates ion conductance and is essential for the proper functioning

> of cells. However, it was not known how an ion imbalance could make

> the lungs an attractive site for bacterial colonisation. Defective

> CFTR leads to the undersialylation of plasma membrane glycoconjugates,

> which promote lung pathology and bacterial colonisation. The

> researchers showed, by imaging with lumenally exposed pH-sensitive

> green fluorescent protein, that dysfunctional CFTR causes excessive

> acidification of the trans-Golgi network in CF lung epithelial cells.

> The features were corrected by incubating CF respiratory epithelial

> cells with weak bases.

>

> If these findings are further substantiated, the researchers see the

> potential for a new approach to treatment. `We already have ion pump

> inhibitors and antacids for treating heartburn', says Vojo Deretic

> (University of New Mexico). `If we can design similar compounds to go

> to the lungs, we might have a simple solution to greatly improve the

> health of CF patients.'

[Guest guest]

Hi,

if those scientists only could come up with a solution instead of developing

new theories all the time :-(

But then I remind myself of the motto of another CF-dad that sounded like

" Every night while you are asleep someone on this earth is working on a cure

for CF "

Peace

Torsten, dad of Fiona 6wcf

e-mail: torstenkrafft@...

> Cystic fibrosis: a new theory

> Scientists have proposed a new theory for the infections that are

> found in cases of cystic fibrosis [5]. The researchers, at the

> University of Michigan Medical School (Ann Arbor, MI, USA) and the

> University of New Mexico Health Sciences Centre (Albuquerque, NM,

> USA), showed that the gene which is mutated in cystic fibrosis (CF)

> can alter the internal chemistry of lung cells, making them more

> susceptible to certain pathogenic bacteria.

>

> `This research proposes a novel explanation for why lung infections

> are so persistent in CF,' said at the National

> Institute of Allergy and Infectious Diseases (NIAID), which funded the

> study.

>

> Cystic fibrosis causes chronic respiratory infections and sufferers

> ultimately die from a build-up of Pseudomonas aeruginosa, which rarely

> causes disease in healthy humans. The CF gene, known since 1989,

> encodes for the CF transmembrane conductance regulator (CFTR), which

> regulates ion conductance and is essential for the proper functioning

> of cells. However, it was not known how an ion imbalance could make

> the lungs an attractive site for bacterial colonisation. Defective

> CFTR leads to the undersialylation of plasma membrane glycoconjugates,

> which promote lung pathology and bacterial colonisation. The

> researchers showed, by imaging with lumenally exposed pH-sensitive

> green fluorescent protein, that dysfunctional CFTR causes excessive

> acidification of the trans-Golgi network in CF lung epithelial cells.

> The features were corrected by incubating CF respiratory epithelial

> cells with weak bases.

>

> If these findings are further substantiated, the researchers see the

> potential for a new approach to treatment. `We already have ion pump

> inhibitors and antacids for treating heartburn', says Vojo Deretic

> (University of New Mexico). `If we can design similar compounds to go

> to the lungs, we might have a simple solution to greatly improve the

> health of CF patients.'