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Topic: Cystic fibrosis transmembrane conductance regulator

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	Human protein: P13569 - Cystic fibrosis transmembrane conductance regulator (CFTR) (cAMP- dependent chloride channel) ...
		A new missense mutation (R1283M) in exon 20 of the cystic fibrosis transmembrane conductance regulator gene.
		A new missense mutation G1249E in exon 20 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
		A missense mutation (F87L) in exon 3 of the cystic fibrosis transmembrane conductance regulator gene.
	harvester.embl.de /harvester/P135/P13569.htm (2042 words)

	Cystic fibrosis: Encyclopedia of Genetic Disorders
		Cystic fibrosis (CF) is an inherited disease that affects the lungs, digestive system, sweat glands, and male fertility.
		Cystic fibrosis is a genetic disease, meaning it is caused by a defect in the person's genes.
		The most severe effects of cystic fibrosis are seen in two body systems: the gastrointestinal (digestive) system and the respiratory tract, from the nose to the lungs.
	health.enotes.com /genetic-disorders-encyclopedia/cystic-fibrosis (4154 words)

	Cystic Fibrosis -- Biotechnology Encyclopedia
		Cystic fibrosis (CF) was first described as a disease in the late 1930s.
		Approximately one in every 25 people of European descent is a carrier for the cystic fibrosis gene, having one normal gene and one CF gene.
		Lap Tsu Chui on the seventh chromosome of the
	www.pipelinedrugs.com /biotechnology_encyclopedia/cystic_fibrosis.htm (949 words)

	CFTR - cystic fibrosis transmembrane conductance regulator, ATP-binding cassette (sub-family C, member 7) (Site not responding. Last check: 2007-09-10)
		Although Cystic fibrosis transmembrane conductance regulator (CFTR) has been shown to regulate the activity of NHE3, the potential reciprocal interaction of NHE3 to modulate the protein kinase A (PKA)-dependent regulation of CFTR in epithelial cells is still unknown.
		A cystic fibrosis (CF) mouse expressing the human mucin MUC1 transgene (CFM) reverted the CF/Muc1(-/-) phenotype (little mucus accumulated in the intestine) to that of CF mice expressing mouse Muc1, which exhibited increased mucus accumulation.
		Human cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in transgenic mice under the control of transcriptional elements derived from the human surfactant protein C (SP-C) gene.
	www.ihop-net.org /UniPub/iHOP/gg/87149.html (6521 words)

	Yet Another Role for the Cystic Fibrosis Transmembrane Conductance Regulator -- Widdicombe 22 (1): 11 -- American ...
		Cystic fibrosis decreases the apical membrane chloride permeability of monolayers cultured from cells of tracheal epithelium.
		Cystic fibrosis transmembrane conductance regulator-dependent regulation of epithelial inducible nitric oxide synthase expression.
		Mutant cystic fibrosis transmembrane conductance regulator inhibits acidification and apoptosis in C127 cells: possible relevance to cystic fibrosis.
	ajrcmb.atsjournals.org /cgi/content/full/22/1/11 (2004 words)

	Choi JY, et al. Cl-Dependent HCO3- Transport by Cystic Fibrosis Transmembrane Conductance Regulator. JOP. J Pancreas ...
		Cystic fibrosis (CF) affects the function of multiple organs, including the pancreas, intestine and the respiratory tract [1].
		The discovery that Cl conductance is impaired in CF [18, 19] and that the CFTR gene codes for a cAMP-regulated Cl channel [8] led to the paradigm that mutations in CFTR that inhibit Cl channel activity, lead to inhibition of transepithelial fluid and electrolyte transport and CF [1, 2, 3, 4, 5, 6, 7].
		Cystic fibrosis transmembrane conductance regulator currents in guinea pig pancreatic duct cells: inhibition by bicarbonate ions.
	www.joplink.net /prev/200107/16.html (1539 words)

	Geometry.Net - Health_Conditions: Cystic Fibrosis
		Seeking the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease.
		Patrick L. Bufi, N.D. Abstract Introduction Cystic fibrosis (CF) is the most common lethal autosomal recessive disease found in the Caucasian population, with a frequency of approximately one in 2,500 births.
		The primary CF defect is expressed as altered ion transport via the cystic fibrosis transmembrane conductance regulator (CFTR), which is the protein regulating cyclic-AMP-mediated chloride conductance at the apical membranes of secretory epithelia.
	www5.geometry.net /health_conditions/cystic_fibrosis.html (1810 words)

	Cystic Fibrosis - Definition, Description, Demographics, Causes and symptoms, Diagnosis, Treatment, Prognosis, ...
		Because of better and earlier treatment, a person born in 2004 with CF is expected, on average, to live to age 40.
		Adults with a family history of cystic fibrosis may obtain a genetic test of their carrier status for purposes of family planning.
		Cystic fibrosis results when a person has two defective copies of the CFTR gene.
	www.healthofchildren.com /C/Cystic-Fibrosis.html (4278 words)

	Gray MA, et al. Functional Interactions of HCO3- with Cystic Fibrosis Transmembrane Conductance Regulator. JOP. J ...
		Disruption of normal cystic fibrosis transmembrane conductance regulator- (CFTR)-mediated Cl transport is associated with cystic fibrosis (CF).
		It is well established that cystic fibrosis transmembrane conductance regulator (CFTR) transports chloride ions in a variety of epithelial tissues.
		Cystic fibrosis transmembrane conductance regulator currents in guinea-pig pancreatic duct cells and their inhibition by bicarbonate ions.
	www.joplink.net /prev/200107/10.html (1641 words)

	Permeation through the CFTR chloride channel -- McCarty 203 (13): 1947 -- Journal of Experimental Biology
		Cystic fibrosis transmembrane conductance regulator (CFTR) confers glibenclamide sensitivity to outwardly rectifying chloride channel (ORCC) in Hi-5 insect cells.
		Yamazaki, J. and Hume, J. Inhibitory effects of glibenclamide on cystic fibrosis transmembrane regulator, swelling-activated and Ca2+-activated Clchannels in mammalian cardiac myocytes.
		Gating of cystic fibrosis transmembrane conductance regulator chloride channels by adenosine triphosphate hydrolysis: Quantitative analysis of a cyclic gating scheme.
	jeb.biologists.org /cgi/content/refs/203/13/1947 (3109 words)

	Cystic Fibrosis Transmembrane Conductance Regulator Degradation Depends on the Lectins Htm1p/EDEM and the Cdc48 Protein ...
		Cystic Fibrosis Transmembrane Conductance Regulator Degradation Depends on the Lectins Htm1p/EDEM and the Cdc48 Protein Complex in Yeast -- Gnann et al.
		Chiang, H.L., and Schekman, R. Regulated import and degradation of a cytosolic protein in the yeast vacuole.
		Ward, C.L., and Kopito, R.R. Intracellular turnover of cystic fibrosis transmembrane conductance regulator.
	www.molbiolcell.org /cgi/content/full/15/9/4125 (6023 words)

	Energy Citations Database (ECD) - Energy and Energy-Related Bibliographic Citations
		Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis
		Reports of familial occurrence of ABPA and increased incidence in CF patients suggest a possible genetic basis for the disease.
		To test this possibility, the entire coding region of the cystic fibrosis transmembrane regulator (CFTR) gene was analyzed in 11 individuals who met strict criteria for the diagnosis of ABPA and had normal sweat electrolytes ({le}40 mmol/liter).
	www.osti.gov /energycitations/product.biblio.jsp?osti_id=443734 (284 words)

	Respiratory Research \| Full text \| Glycosylation and the cystic fibrosis transmembrane conductance regulator
		Cystic Fibrosis Center and Department of Pediatrics, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Abramson Pediatric Research Center, Philadelphia, Pennsylvania, USA
		The cystic fibrosis transmembrane conductance regulator (CFTR) has been known for the past 11 years to be a membrane glycoprotein with chloride channel activity.
		Denning GM, Ostedgaard LW, Welsh M: Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia.
	respiratory-research.com /content/2/5/276 (2543 words)

	Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts
		Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts
		Effective and specific inhibition of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel in epithelia has long been needed to better understand the role of anion movements in fluid and electrolyte transport.
		X F. Wang, M M. Reddy, and Paul M. Quinton, "Effects of a new cystic fibrosis transmembrane conductance regulator inhibitor on Cl- conductance in human sweat ducts" (2004).
	repositories.cdlib.org /postprints/370 (341 words)

	Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 ...
		Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 membrane-spanning segment -- Cheung and Akabas 70 (6): 2688 -- Biophysical Journal
		Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 membrane-spanning segment
		Cystic Fibrosis-associated Mutations at Arginine 347 Alter the Pore Architecture of CFTR.
	biophysj.org /cgi/content/abstract/70/6/2688?maxtoshow=&HITS=10&... (1408 words)

	Recent Literature Reviews About Cystic Fibrosis Transmembrane Conductance Regulator
		Studies showing that the abnormality in chloride flux could be corrected by transfection of wild-type cystic fibrosis transmembrane conductance regulator (CFTR) complimentary deoxyribonucleic acid (cDNA) have led to gene therapy trials on both sides of the Atlantic.
		Treatment options for cystic fibrosis have developed rapidly since discovery of the cystic fibrosis gene over a decade ago.
		As approximately 90% of cystic fibrosis patients have a version of this deltaF508 CFTR protein, if a treatment existed to improve its function, this potential treatment could help a considerable number of patients.
	opal.msu.montana.edu /cftr/recent_literature_reviews.htm (2422 words)

	Howstuffworks "How Cells Work"
		Cystic fibrosis - In cystic fibrosis, the gene that manufactures the protein called cystic fibrosis transmembrane conductance regulator is damaged.
		The defect (or mutation) found in the gene on chromosome 7 of persons with cystic fibrosis causes the production of a protein that lacks the amino acid phenylalanine.
		Chronic cough, recurrent pneumonia, and the progressive loss of lung function are the major manifestations of lung disease, which is the most common cause of death of persons with cystic fibrosis.
	www.howstuffworks.com /cell.htm/printable (5734 words)

	Search Tuna Report for cystic fibrosis (Site not responding. Last check: 2007-09-10)
		Cystic fibrosis is the most common cause of chronic lung disease in children and young adults, and the most common fatal hereditary disorder affecting Caucasians in the US.
		Cystic Fibrosis Is A Chronic, Frequently Fatal, Progressive, Genetically Disease Of The Body's Mucus Glands....
		Cystic Fibrosis Foundation The Mission Of The Cystic Fibrosis Foundation Is To Assure The Development Of The Means To Cure And Control Cystic Fibrosis And To Improve The Quality Of Life For Those With The Disease....
	www.searchtuna.com /ftlive2/3151.html (1962 words)

	Amazon.com: The Cystic Fibrosis Transmembrane Conductance Regulator (Molecular Biology Intelligence Unit): Books: Kevin ... (Site not responding. Last check: 2007-09-10)
		The Cystic Fibrosis Transmembrane Conductance Regulator addresses a select series of `hot' topics that relate to the function of CFTR, and the links between CFTR dysfunction and human disease (i.e., cystic fibrosis).
		Given the general interest in CFTR, this collection will appeal to a broad readership with interests in CFTR, cystic fibrosis, ion channels and ABC transporters.
		Cystic Fibrosis treatment info and resources provided by Genentech.
	www.amazon.com /exec/obidos/tg/detail/-/0306478374?v=glance (589 words)

	Adenosine Triphosphate-dependent Asymmetry of Anion Permeation in the Cystic Fibrosis Transmembrane Conductance ...
		Adenosine Triphosphate-dependent Asymmetry of Anion Permeation in the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel
		The cystic fibrosis transmembrane conductance regulator (CFTR) forms a tightly regulated channel that mediates the passive
		Perturbation of the Pore of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibits Its ATPase Activity
	www.jgp.org /cgi/content/abstract/111/4/601 (1199 words)

	The Cystic Fibrosis Transmembrane Conductance Regulator (Site not responding. Last check: 2007-09-10)
		The first four chapters address issues related to the function and regulation of the CFTR channel and its role in controlling fluid and electrolyte transport across epithelial tissues.
		The Cystic Fibrosis Transmembrane Conductance Regulator provides a glimpse into some of these properties and insights into several of the remaining questions regarding the biology and pathophysiology of this fascinating and critically important ion channel.
		The CFTR Gene and Genetics of Cystic Fibrosis
	www.vonl.com /chips/cystic.htm (247 words)

	Cystic fibrosis transmembrane conductance regulator:
		CFTR also regulates the activity of other membrane proteins including the epithelial Na channel and outwardly rectifying chloride channel.
		1 Pathogenesis and clinical manifestations of cystic fibrosis (Cuthburt AW, 1996)
		As indicted in figure 2, the molecule consists of five distinct domains - two membrane spanning domains (MSDs) each containing six transmembrane a helices, two cystolic nuceotide binding folds (NBFs) and a cystolic regulatory ‘R’ domain.
	www.portfolio.mvm.ed.ac.uk /studentwebs/session2/group5/cftr.htm (169 words)

	Title page for ETD etd-06102003-144147
		The cystic fibrosis transmembrane conductance regulator (CFTR) is one of the most studied membrane protein models because of its clear clinical significance.
		Mutations within the CFTR gene lead to cystic fibrosis, the most common autosomal recessive genetic disorder in the Caucasian population.
		CFTR, a large 160 kDa glycoprotein, is a chloride ion channel in the ABC superfamily of transporter proteins.
	scholar.lib.vt.edu /theses/available/etd-06102003-144147 (272 words)

	CFTR (Cystic fibrosis transmembrane regulator) antibodies from Research Diagnostics Inc
		Backgorund: Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel.
		The immunizing sequence is identical in monkey and rabbit, has 11/12 identical amino acids in sheep, cow and frog, 10/12 identify in mouse and 9/12 in rat.fusion protein corresponding to residues 1197-1480 of human cystic fibrosis transmembrane conductance regulator (CFTR).
		Antigen: fusion protein corresponding to residues 1197-1480 of human cystic fibrosis transmembrane conductance regulator (CFTR).
	www.researchd.com /miscabs/cftrab.htm (1080 words)

	Cystic Fibrosis Transmembrane Conductance Regulator. STRUCTURE AND FUNCTION OF AN EPITHELIAL CHLORIDE CHANNEL -- Akabas ...
		In addition to its function as a Cl channel, CFTR acts as a regulator of other channels and transporters (26).
		CFTR has an important role in epithelial Cl transport both as a Cl channel and as a regulator of other channels and transporters.
		The abbreviations used are: CFTR, cystic fibrosis transmembrane conductance regulator; CF, cystic fibrosis; DIDS, 4,4'-diisothiocyanostilbene-2,2'-disulfonic acid; MDR, multiple drug resistance protein; NBD, nucleotide-binding domain; PKA, cAMP-dependent protein kinase; PKC, protein kinase C; ER, endoplasmic reticulum.
	www.jbc.org /cgi/content/full/275/6/3729 (3695 words)

	Cystic Fibrosis Transmembrane Conductance Regulator: Physical Basis for Lyotropic Anion Selectivity Patterns -- Smith ...
		Cystic Fibrosis Transmembrane Conductance Regulator: Physical Basis for Lyotropic Anion Selectivity Patterns -- Smith et al.
		Cystic Fibrosis Transmembrane Conductance Regulator: Physical Basis for Lyotropic Anion Selectivity Patterns
		conduction path of a narrow region in which the residues that
	www.jgp.org /cgi/content/full/114/6/799 (8493 words)

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