Limb-girdle Muscular Dystrophy Type 2A Can Result from Accelerated Autoproteolytic Inactivation of Calpain 3.

Loss-of-function mutations in calpain 3 have been shown to cause limb-girdle muscular dystrophy type 2A (LGMD2A), an autosomal recessive disorder that results in gradual wasting of the muscles of the hip and shoulder areas. Due to the inherent instability of calpain 3, recombinant expression of the full-length enzyme has not been possible, making in vitro analysis of specific LGMD2A-causing mutations difficult. However, because calpain 3 is highly similar in amino acid sequence to calpain 2, the recently solved crystal structure of full-length, Ca2+-bound, calpastatin-inhibited rat calpain 2 has allowed us to model calpain 3 as a Ca2+-bound homodimer. The model revealed three distinct areas of the enzyme that undergo a large conformational change upon Ca2+-binding. Located in these areas are several residues that undergo mutation to cause LGMD2A. We investigated the in vitro effects of six of these mutations by making the corresponding mutations in rat calpain 2. All six mutations examined in this study resulted in a decrease in enzyme activity. All but one of the mutations caused an increased rate of autoproteolytic degradation of the enzyme as witnessed by SDS-PAGE, indicating the decrease in enzyme activity is caused, at least in part, by an increase in the rate of autoproteolytic degradation. The putative in vivo effects of these mutations on calpain 3 activity are discussed with respect to their ability to cause LGMD2A.

Get your 10 a day! The Nursing Care Standards for Patient Food in Hospital (PDF 1.92 MB)

Standards to improve the nutritional care and food experience of patients in hospital.

Appendices for Complex Needs - The Nursing Response to Children and Young People with Complex Physical Healthcare Needs (PDF 708 KB)

Appendices to Complex Needs Report

Complex Needs - The Nursing Response to Children and Young People with Complex Physical Healthcare Needs (PDF 832 KB)

Departmental review of nursing services in order to ensure that they are facilitated to fully support and respond to children with complex needs and their families, and for them to work in partnership with other professions and agencies.

Modernising Nursing Careers : Setting the Direction (PDF 278 KB)

This report sets the direction for modernising nursing careers.

Higher education as formation of the self and citizenship: an ethnography of the University Students from Amman, Jordan

My interest in higher education and citizenship in the Middle East at large and in Jordan in particular is fostered by some of the reflections Eickelman proposed (1992). Being a quite recent phenomenon, intimately linked with the more general topic of state formation it seemed to me more suitable to study it in a little country with a recent history (a field study left almost unexplored until now as far as Jordan is concerned, to the best of my knowledge, since Antoun 1994 focuses on the migration as a quest for higher education). The process of state formation in Jordan is quite studied. I thus intended to study the higher education policies as an attempt both to create a national citizenry and more recently as a way of controlling the more problematic part of the population (youth, which constitutes more than the double of the population. See UNDP and Ministry of Planning 2000). How do the young students enter the university system, and in which way does this system work? How is this system designed, in order to retain social control of the students (since they are usually perceived to be a factor of social and political instability, as in Iran or in Egypt)? Is there any significant difference between different faculties? And if so, why? My conclusions at this stage are that the university system is an integral part of the survival of the regime. The system works quite well, and Jordan has one of the best educational position in the region. Yet there are important distinctions to be made: the access to the better faculties is socially selective while the less valued faculties are left to the poorer and less wealthy youth. This results in a different treatment of the students and of the courses that I analysed. In the better faculties the teaching standards are quite high, and the relationship between professors and students is almost on a same-level base, while in the less privileged faculties the opposite is true. Thus we can observe a concrete politics of divide et impera intended to split the youth in two. For the more privileged there are some freedoms, both within and outside classes, designed I guess at forging them as autonomous individuals. On the opposite the less privileged are kept under tight control, even if also these students are a privileged category among youth at large.

District Nursing Services in Northern Ireland Follow Up Regional Report (PDF 1080 KB)

District Nursing Services in Northern Ireland Follow Up Regional Report

Free Nursing Assessment Tool (PDF 159 KB)

A component of the Nursing Needs Assessment Tool. This document is the format for the Free Nursing Assessment Review. First review should be carried out within 3 months and annually thereafter.

Nursing Needs Assessment Tool (PDF 255 KB)

This assessment tool is designed to assess the registered nursing needs of an older person needing long-term care.

Nursing Needs Assessment Tool User Guide 2006 (PDF 207 KB)

The user guide describes how to undertake an assessment using the nursing needs assessment tool.

Development of Public Health Nursing within Public Health Nursing in the Poleglass Twinbrook and Lagmore Areas of Down Lisburn Trust (PDF 424 KB)

Project evaluation report on the development of public health nursing within children's services in areas of Down Lisburn Trust. Part of the Department's redesign of community nursing project

Evaluation of Community Nursing Project : Moving Professional Boundries From A Stoma Care Nursing Service To A Colorectal Nursing Service (PDF 3607 KB)

Foyle HSS Trust's Evaluation of community nursing project. Part of the Department's redesign of community nursing project.

Homefirst Community Trust - Community Nursing Project - Glenfield Project (PDF 345 KB)

Homefirst Community Trust's final report on the Glenfield Project. Part of the Department's redesign of community nursing project

South & East Belfast Community Nursing Project - Primary Care Integrated Nursing Model (PDF 524 KB)

South & East Belfast H&SS Trust's final report on Primary Care Integrated Nursing Model. Part of the Department's redesign of community nursing project

Sperrin Lakeland Health & Social Care Trust Community Nursing Project - Evaluation Report on Establishment of a treatment Room Service Bureau (PDF 316 KB)

Sperrin Lakeland Trust's final report on the establishment of a treatment room service bureau. Part of the Department's redesign of community nursing project