scholarly journals Maintaining mental health and function for the long run in cystic fibrosis

2016 ◽  
Vol 51 (S44) ◽  
pp. S71-S78 ◽  
Author(s):  
Beth A. Smith ◽  
Anna M. Georgiopoulos ◽  
Alexandra L. Quittner
2020 ◽  
Vol 19 ◽  
pp. S49
Author(s):  
L. Backström Eriksson ◽  
C. Laine ◽  
I. de Monestrol ◽  
A. Lindblad ◽  
E. Bergenmar-Ivarsson ◽  
...  

1999 ◽  
Vol 79 (1) ◽  
pp. S23-S45 ◽  
Author(s):  
DAVID N. SHEPPARD ◽  
MICHAEL J. WELSH

Sheppard, David N., and Michael J. Welsh. Structure and Function of the CFTR Chloride Channel. Physiol. Rev. 79 , Suppl.: S23–S45, 1999. — The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique member of the ABC transporter family that forms a novel Cl− channel. It is located predominantly in the apical membrane of epithelia where it mediates transepithelial salt and liquid movement. Dysfunction of CFTR causes the genetic disease cystic fibrosis. The CFTR is composed of five domains: two membrane-spanning domains (MSDs), two nucleotide-binding domains (NBDs), and a regulatory (R) domain. Here we review the structure and function of this unique channel, with a focus on how the various domains contribute to channel function. The MSDs form the channel pore, phosphorylation of the R domain determines channel activity, and ATP hydrolysis by the NBDs controls channel gating. Current knowledge of CFTR structure and function may help us understand better its mechanism of action, its role in electrolyte transport, its dysfunction in cystic fibrosis, and its relationship to other ABC transporters.


2018 ◽  
Vol 28 (03) ◽  
pp. 254-257 ◽  
Author(s):  
Sinan Guloksuz ◽  
Jim van Os

AbstractThere had been a long way to go before we felt comfortable about even discussing the issues revolving around the concept of ‘schizophrenia’, let alone reckoning on mere semantic revision. In this editorial, we aim to extend our discussion on the reasons behind the slow death of the concept of ‘schizophrenia’ and the benefits of changing the name and embracing a spectrum approach with an umbrella psychosis spectrum disorder (PSD) category (similar to autism spectrum disorder) that goes further than a mere semantic revision. We attempted to cover the topic of the renaming by providing five most pertinent points categorised under five domains: reasons, signals, challenges, promises and steps for the change. Admittedly, even a modest revision, such as classifying all psychotic disorder categories under an umbrella category of PSD, and abolishing the term schizophrenia requires careful deliberation and some effort in the beginning, but the revision is well worth the effort considering the benefits in the long run. Renaming a particular form of mental suffering should be accompanied by a broader debate of the entire diagnosis-evidence-based-practice (EBP)-symptom-reduction model as the normative factor driving the content and organisation of mental health services that may be detached from patients’ needs and reality, overlooks the trans-syndromal structure of mental difficulties, appraises the significance of the technical features over the relational and ritual components of care, and underestimates the lack of EBP group-to-individual generalisability. Individuals may make great strides in attaining well-being by accommodating to living with mental vulnerabilities through building resilience in the social and existential domains. Changing the name and the concept of ‘schizophrenia’, which goes beyond a mere semantic revision, may become the first step that allows catalysation of the process of modernising psychiatric science and services worldwide.


Author(s):  
Kathryn L. Behrhorst ◽  
Robin S. Everhart ◽  
Michael S. Schechter

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