A new model of cystic fibrosis pathology: Lack of transport of glutathione and its thiocyanate conjugates

Childers, Melanie; Eckel, George; Himmel, A; Caldwell, J E

doi:10.1016/j.mehy.2006.06.020

Cited by 46 publications

(32 citation statements)

References 103 publications

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“…The "Prediction on log-odds"-table represents the topology state winners (see (6)) followed by the "F-measures"- with respect to the three topology states. The highest logodd sum leads to the topology state winner (see (6)). Crossvalidation was performed by excluding the evaluation set of motifs from the training motif set, which was used to generate the look-up log-odd values.…”

Section: High Propensitymentioning

confidence: 99%

“…The "Prediction on log-odds"-table represents the topology state winners (see (6)) followed by the "F-measures"- for each motif generated from TMHMM information. These statistics are listed under the "TMHMM prediction"-table heading and the right of it followed by our predicted (see (6)) information. The prediction quality is determined by the respective F-values.…”

Section: Evaluation Of the Prediction Accuracymentioning

confidence: 99%

“…On the other hand, biological processes can be comprehended as a dynamically fluctuating system, whereby the biological role of the unknown membrane protein can be defined more precisely [3,4]. Accordingly, destabilisation of the three-dimensional structure of a membrane protein caused by mutations or ligand interactions are triggers for numerous diseases, for example, diabetes insipidus, cystic fibrosis, hereditary deafness and retinitis pigmentosa [5][6][7].…”

Section: Introductionmentioning

confidence: 99%

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Structure Topology Prediction of Discriminative Sequence Motifs in Membrane Proteins with Domains of Unknown Functions

2013

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Motivation. Membrane proteins play essential roles in cellular processes of organisms. Photosynthesis, transport of ions and small molecules, signal transduction, and light harvesting are examples of processes which are realised by membrane proteins and contribute to a cell's specificity and functionality. The analysis of membrane proteins has shown to be an important part in the understanding of complex biological processes. Genome-wide investigations of membrane proteins have revealed a large number of short, distinct sequence motifs. Results. The in silico analysis of 32 membrane protein families with domains of unknown functions discussed in this study led to a novel approach which describes the separation of motifs by residue-specific distributions. Based on these distributions, the topology structure of the majority of motifs in hypothesised membrane proteins with unknown topology can be predicted. Conclusion. We hypothesise that short sequence motifs can be separated into structure-forming motifs on the one hand, as such motifs show high prediction accuracy in all investigated protein families. This points to their general importance in -helical membrane protein structure formation and interaction mediation. On the other hand, motifs which show high prediction accuracies only in certain families can be classified as functionally important and relevant for family-specific functional characteristics.

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Section: High Propensitymentioning

confidence: 99%

Section: Evaluation Of the Prediction Accuracymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Structure Topology Prediction of Discriminative Sequence Motifs in Membrane Proteins with Domains of Unknown Functions

2013

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“…It is suggested that the antioxidant action of zinc and vitamin C may affect the flux of glutathione across cells and modify the rheology of sputum in cystic fibrosis. 18,19 Data on zinc supplementation among children with cystic fibrosis are limited to 4 studies. [6][7][8][9] Safai-Kutti et al, 9 in their placebo-controlled double-blind crossover study in children with cystic fibrosis (N ϭ 13, age 2 y, 3 months to 19 y, 1 month), gave zinc supplementation and placebo to the subjects for 6 months.…”

Section: Discussionmentioning

confidence: 99%

Zinc Supplementation for One Year Among Children with Cystic Fibrosis Does Not Decrease Pulmonary Infection

Sharma¹,

Lodha²,

Shastri³

et al. 2015

Respir Care

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BACKGROUND: Children with cystic fibrosis may have a deficiency of micronutrients, including zinc, which may affect their susceptibility to infections. There is a paucity of data on zinc supplementation among children with cystic fibrosis. We hypothesized that a pharmacologic dose of zinc administered daily for 12 months would reduce the need for antibiotics by 50%. METHODS: This double-blind randomized placebo-controlled trial was conducted among children with cystic fibrosis to assess the effect of zinc supplementation on the need for antibiotics and pulmonary function tests. The children, age 5-15 y, of either sex, received either 30-mg zinc tablets or similar looking placebo tablets daily in addition to standard care. They were followed up every month for a period of 12 months and whenever they had pulmonary exacerbations. Their serum zinc was estimated at baseline and at 12 months of enrollment. During each visit, the children underwent a pulmonary function test and sputum culture. RESULTS: Of a total of 43 children screened, 40 were enrolled, and of them, 37 completed the study. The median (interquartile range) number of days of the administration of antibiotics over 12 months of follow-up among the children receiving zinc was 42 (14 -97) d. In the placebo group, it was 38 (15-70) d (P ‫؍‬ .79). There were no significant differences in the percent-of-predicted FEV 1 or change in FEV 1 values at 12 months (P ‫؍‬ .44). The number of children in whose respiratory specimens Pseudomonas was isolated was similar for the 2 groups at different time intervals. The adverse events reported were similar in the 2 groups. CONCLUSION: We did not find any significant difference in the need for antibiotics, pulmonary function tests, hospitalization, colonization with Pseudomonas, or the need for antibiotics for children with cystic fibrosis receiving zinc supplementation of 30 mg/d.

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“…Defensins Impaired activity Goldman MJ et al 1997, Bals R et al 2001 Surfactant proteins Decreased or inactive Hartl D, Griese M 2006, Meyer KC et al 2000, Noah TL et al 2003 Antioxidants Reduced glutathione availability in airways Gao L et al 1999, Roum JH et al 1993, Day BJ 2005, Childers M et al 2007 Moss RB et al1996, Mueller C et al2010 IL-6 4 Increased secretion Black HR et al 1998, Becker MN et al 2004, Andersson C et al 2007, Vandivier RW et al 2009 Increased secretion Bonfield TL et al 1995, Black HR et al 1998, Becker MN et al 2004, Vandivier RW et al 2009 Altered secretion Bonfield TL et al 1995, Moss RB et al 1996, Armstrong DS et al 2005 Increased secretion Mueller C et al2010 IL-17 8 Increased secretion McAllister F et al2005, Tan HL et al2011 TNF- 9 Increased secretion Bonfield TL et al 1995, Andersson C et al 2007 Increased secretion Brennan S et al 2009 MCP-1 11 Increased secretion Brennan S et al 2009 Notes: 1 IFN-( interferon-gamma); 2 IL-1(interleukin-1 beta), 3 IL-4 (interleukin-4), 4 IL-6 (interleukin-6), 5 IL-8 (interleukin-8), 6 IL-10 (interleukin-10), 7 IL-13 (interleukin-13), [8][9][10][11][12][13][14][15][16][17], 9 TNF-...…”

Section: Mediator Abnormality Referencementioning

confidence: 99%

Immune Dysfunction in Cystic Fibrosis

Xu¹,

Worgall²

2012

Cystic Fibrosis - Renewed Hopes Through Research

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A new model of cystic fibrosis pathology: Lack of transport of glutathione and its thiocyanate conjugates

Cited by 46 publications

References 103 publications

Structure Topology Prediction of Discriminative Sequence Motifs in Membrane Proteins with Domains of Unknown Functions

Structure Topology Prediction of Discriminative Sequence Motifs in Membrane Proteins with Domains of Unknown Functions

Zinc Supplementation for One Year Among Children with Cystic Fibrosis Does Not Decrease Pulmonary Infection

Immune Dysfunction in Cystic Fibrosis

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