Welcome to the Niemann-Pick Disease Group (UK)

The Peter Carlton Jones Memorial Award 2012 - Up to £1000.00

It is proposed that an annual award of up to one thousand pounds (£) will be made to an individual who is engaged in either research or teaching or treatment or care, within the public or private sectors in the United Kingdom.

The award to be granted in response to the submission of a ‘research project’ (not a literature review) which provides an original contribution to the scientific or public understanding of the Niemann-Pick diseases and/or their treatment or cure.

The applicant’s submission of a completed project should be in the form of an abstract, of one side of A4 paper (~ 600 words), which includes clear statements on the work undertaken, methodology, ‘findings’ and principal features in regard to the nature of the project, as referred to above.

The decision on the award will be determined by an Award Committee of the Niemann-Pick Disease Group (UK). The Committee comprising; the Group’s Chairman, the Medical Advisor, the Trustee with a research remit, plus the Clinical Nurse Specialist for Niemann-Pick Diseases.

Applicants are invited to submit for the 2012 Award, with a view to the Award Committee short listing and possibly seeking further information and/or a project report, prior to a final decision being made.

Submissions to: Toni Mathieson

Executive Director

Niemann-Pick Disease Group (UK)

Suite 2, Vermont House

Concord, Washington NE37 2SQ

E-mail: niemann-pick@zetnet.co.uk

Tel: 0191 415 0693

Closing date for submissions: 30th June 2012

It is envisaged that the successful applicant will be invited to the Annual Family Conference of the Niemann-Pick Disease Group (UK), to be held in September 2012, to formally receive the award.

The Award has been established in memory of Peter Carlton Jones a former Trustee of the Niemann-Pick Disease Group (UK).

The Peter Carlton Jones Memorial Award 2010 - The Winner

After careful consideration of the submissions received, which were once again of a very high standard, the Peter Carlton Jones Memorial Award Committee agreed to award the 2010 prize to Sarah Pressey, a young scientist with an interest in how the brain is affected by Niemann-Pick Disease. Sarah is in the final stages of writing up her PhD in which she has been characterizing what happens in the brain in Niemann-Pick Type C (NPC). Her PhD has been at The Institute of Psychiatry, King’s College London in the lab of Dr Jonathan Cooper, with additional supervision from Prof Fran Platt of Oxford University.

Sarah writes:

“Niemann-Pick Type C (NPC) disease is caused by mutations in either the NPC1 or NPC2 genes, but we don’t understand how the defects in the proteins they code for has such a devastating effect upon the brain. To investigate this we have systematically examined the NPC brain to determine what brain regions and types of cells are affected. To do this we have used a mouse model of NPC (Npc1-/- mice), which has the same genetic defect that causes the human disease. First, we examined the “activation” of support cells (glial cells) of the brain; in the healthy brain these glial cells provide the correct environment for the neurons to function properly, but in the diseased brain, activation of glial cells can be an indication of damage to the brain. We have found that these glial cells become activated early in disease progression in the Npc1-/- mice brain, particularly in pathways that process sensory information. Following this, we found that interconnected sensory pathways are affected in the Npc1-/- mice brain. By examining very young mice, we have found that neurons are lost in these pathways early in disease progression, long before the mice become symptomatic. Our study has identified, firstly specific brain regions that are affected early in pathogenesis and secondly, pathological events that occur in the brain as a result of loss of NPC1 protein function. This knowledge has increased our understanding of the impact that NPC disease has upon the brain and will help inform future therapeutic approaches.”

The Peter Carlton Jones Memorial Award 2009 - The Winners

After careful consideration of the submissions received, which were once again of a very high standard, the Peter Carlton Jones Memorial Award Committee agreed to jointly award the 2009 prize to Dr Barry Boland and Stephen Knight.

Read Barry Boland's submission

Read Stephen Knight's submission

The Peter Carlton Jones Memorial Award 2008

The winner of the 2008 Peter Carlton Jones Memorial Award was Alexander Lewis.

Read Alexander's submission

The Peter Carlton Jones Memorial Award 2007

In 2007, the Peter Carlton Jones Memorial Award Committee agreed to award the prize jointly to two deserving candidates:

Daniel Witter - Read Daniel’s submission

Jinzhi Zhang – Read Jinzhi’s submission

The Peter Carlton Jones Memorial Award 2006

The winner of the 2006 Peter Carlton Jones Memorial Award was Emyr-Lloyd-Evans Read Emyr's submission