Guest guest Posted December 19, 2002 Report Share Posted December 19, 2002 this came in from a friend of mine father of Cameron (2yrs on Dec 17) and Claire 6wks old both wcf Cystic Fibrosis ASCB 2002 - Day 3 - Tuesday 17 December 2002 HYPERLINK " http://download.bmn.com/images/bmn_green_pixel.gif " Report: Cystic fibrosis research turns hairy Investigator: Rosenbaum 17 December 2002 by Damaris Christensen The mutated protein that causes cystic fibrosis is, at least in healthy mice and rabbits, found on cilia, the hair-like structures that project from cells, scientists today reported. Understanding exactly where the protein is located may lead to new ways of looking at the disease, says Yale University researcher Rosenbaum. More than a decade after researchers identified the mutated gene responsible for cystic fibrosis, scientists are still struggling to understand how that mutation leads to the various problems of the disease. Thick, gooey mucus clogs the lungs of people with cystic fibrosis, and chronic infections - typically caused by the usually harmless bacterium called Pseudomonas aeruginosa - cause respiratory failure, which kills most people with CF while they are in their 20s or early 30s. " Cilia in the lung beat and move bacteria and mucus through the lungs, and they seem to beat properly in people with cystic fibrosis, " said Rosenbaum, " so researchers looking into the disease abandoned the field. " Based on studies suggesting cilia have a role in sensing the environment, however, Rosenbaum and his colleagues thought cilia in the lungs may sense fluid and ionic concentrations. The researchers examined whether the protein that is mutated in cystic fibrosis, the cystic fibrosis transmembrane conductance regulator (CFTR), would be present on cilia. Previous research had found that CFTR is located on the upper, or apical, surface of cells lining the lung and trachea, but the studies did not have a fine enough resolution to determine if CFTR was found on the membrane surrounding cilia, or elsewhere on the apical membrane. Rosenbaum and his colleagues developed a novel method for isolating membrane-coated cilia from cells taken from the trachea of rabbits. Used biochemical methods, they determined that at least 80% of the CFTR found in these cells is present on the cell membrane surrounding the cilia. Researchers also used electron microscopy to look at the tracheal cells, and used antibodies to CFTR to determine exactly where the protein is found. Again, the majority of CFTR is present on cilia, Rosenbaum said. Studies in mice thus far corroborate these results in rabbits, he added. CFTR is on the cilia to help cells sense, and regulate, their environment, Rosenbaum suggests. A thin liquid layer coats cells in the lung and trachea, with mucus floating on top. The cilia beat back and forth in this liquid layer to move mucus through the lungs. Previous research has established that CFTR transports chloride in and out of cells, and that, in turn, is linked to sodium transport. When mutated, CFTR cannot properly regulate sodium absorption by lung cells. Without a brake on sodium absorption, some scientists reason, cells keep drawing in sodium, and water - pulled by osmotic pressure - follows. The liquid layer coating the lungs thins as its water is absorbed by cells. The cilia, in turn, cannot beat effectively, and mucus clogs the lungs, preventing bacteria from being normally cleaned out and providing a breeding ground for them. The defect in the CFTR protein may prevent it from getting to the cilia where it belongs, Rosenbaum suggests, though he has yet to test this hypothesis. Without these receptors, he adds, cilia, and thus cells, can't detect whether the water is too salty or too dilute. " This doesn't tell us how the disease actually occurs, " Rosenbaum said, but he thinks researchers should start paying more attention to the possible sensory roles of cilia in cystic fibrosis. Although the work is potentially important, the experiments were done in healthy mice and rabbits, pointed out one expert in the field. Until it has been shown to be applicable in humans, he cautioned, the utility of such work is unclear. HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " ASCB 2002 American Society for Cell Biology HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&pagefile=content.html " Contents HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&pagefile=confsummary.html " Summary HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " Day: HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&pagefile=content.html#sec_day_1 " 1 HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&pagefile=content.html#sec_day_2 " 2 3 HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&pagefile=content.html#sec_day_4 " 4 HYPERLINK " http://download..bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " Day Reports: (Investigator's name) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&fileday=day3&pagefile=story_1.html " More evidence that all life is shaped by cell death (Kenna Mills) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&fileday=day3&pagefile=story_2.html " Stopping DNA synthesis in its tracks (Kip Sluder) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&fileday=day3&pagefile=story_3.html " Watching embryos in action (Sherry Babb and Marrs) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/bmn_side_nav_arrow_brown.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " Cystic fibrosis research turns hairy ( Rosenbaum) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&fileday=day3&pagefile=story_5.html " Myosins offer a view from the tip ( Cheney) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&fileday=day3&pagefile=story_6.html " Actin tails drive organelle movement (a Giardini) HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " Day Profiles: HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&fileday=day3&pagefile=profile_1.html " Wieschaus HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " View all HYPERLINK " http://news.bmn.com/conferences/list/view?fileyear=2002&fileacronyn=ASC B&pagefile=allprofiles.html " Profiles HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://www.ascb.org/ " American Society for Cell Biology Site HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " HYPERLINK " http://download.bmn.com/images/spacer.gif " Printer ready version HYPERLINK " http://news.bmn.com/email2friend?area=conferences2&referer=%2Fconferenc es%2Flist%2Fview%3Ffileyear%3D2002%26fileacronyn%3DASCB%26fileday%3Dday3 %26pagefile%3Dreport_4.html%26type%3Dreport%26num%3D4&e2f.title=Cystic+f ibrosis+research+turns+hairy&e2f.date=17 December 2002&e2f.day=3&e2f.acro=ASCB&e2f.year=2002&e2f.type=report " See also: HYPERLINK " http://reviews.bmn.com/journals/atoz/latest?uid=ADR.bmn02700_0169409x_v 0051i01_01001727 " The nose and paranasal sinuses physiology and anatomy [Advanced Drug Delivery Reviews] N Advanced Drug Delivery Reviews, 2001, 51:1-3:5-19 HYPERLINK " http://reviews.bmn.com/journals/atoz/latest?uid=TCB.etd01320_09628924_v 0012i08_00002331 " Altered trafficking and epithelial cell polarity in disease [Trends in Cell Biology] M Stein and A Wandinger-Ness Trends in Cell Biology, 2002, 12:8:374-381 HYPERLINK " http://reviews.bmn.com/journals/atoz/latest?uid=TIPS.etd01594_01656147_ v0023i11_00002094 " Disease genes: flattery and deception [Trends in Pharmacological Sciences] A Cuthbert Trends in Pharmacological Sciences, 2002, 23:11:504-509 Printer ready version HYPERLINK " http://news.bmn.com/email2friend?area=conferences2&referer=%2Fconferenc es%2Flist%2Fview%3Ffileyear%3D2002%26fileacronyn%3DASCB%26fileday%3Dday3 %26pagefile%3Dreport_4.html%26type%3Dreport%26num%3D4&e2f.title=Cystic+f ibrosis+research+turns+hairy&e2f.date=17 December 2002&e2f.day=3&e2f.acro=ASCB&e2f.year=2002&e2f.type=report " Editor's Choice Links Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells. HYPERLINK " http://research.bmn.com/medline/search/results?keyword_field=fullrec∨ der=rel&keywords=penque_d:au " Penque D HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " Lab Invest2000 Jun 80:6 857-68 HYPERLINK " http://research.bmn.com/medline/search/record?uid=MDLN.20336084 " MEDLINE HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " HYPERLINK " http://research.bmn.com/medline/search/record?uid=MDLN.20336084&rendert ype=full " Full Medline HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " HYPERLINK " http://research.bmn.com/medline/search/related?uid=MDLN.20336084 " Relate d Records HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " HYPERLINK " http://www.sciencedirect.com/science?_ob=GatewayURL&_origin=BioMedNet& " A proteomic analysis of human cilia: identification of novel components. HYPERLINK " http://research.bmn.com/medline/search/results?keyword_field=fullrec∨ der=rel&keywords=ostrowski_le:au " Ostrowski LE HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " Mol Cell Proteomics2002 Jun 1:6 451-65 HYPERLINK " http://research.bmn.com/medline/search/record?uid=MDLN.22160211 " MEDLINE HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " HYPERLINK " http://research.bmn.com/medline/search/record?uid=MDLN.22160211&rendert ype=full " Full Medline HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " HYPERLINK " http://research.bmn.com/medline/search/related?uid=MDLN.22160211 " Relate d Records HYPERLINK " http://news.bmn.com/conferences/list/$/images/spacer.gif " http://www.sciencedirect.com/science?_ob=GatewayURL&_origin=BioMed Net&_urlversi --- Incoming mail is certified Virus Free. Checked by AVG anti-virus system (http://www.grisoft.com). 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