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Profile of Richard Jones
 

Richard Jones

 
Research Assoc. - Kennedy Krieger Institute
 
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Company Name : Kennedy Krieger Institute
 
Company Website : www.kennedykrieger.org
 
Company Address : 707 North Broadway
, Baltimore, MD,
United States,
 
Richard Jones Profile :
Research Assoc. - Kennedy Krieger Institute
 
Richard Jones Biography :

Dr. Richard Jones is a Research Associate in Neurology at the Kennedy Krieger Institute and also at Johns Hopkins University School of Medicine.

Biographical Sketch:

Dr. Jones received a B.Sc. degree from The University of Manchester (UK) in 1985, and a Ph.D. degree also from The University of Manchester in 1989. After obtaining his Ph.D. Dr. Jones spent a year as a Royal Society Fellow at Shinshu Universuty, Matsumoto, Japan. In 1990 he was appointed a Research Fellow in the Department of Surgery, University of Maryland at Baltimore (UMAB). In 1994 Dr. Jones became an Assistant Professor in Surgery at UMAB. He joined the Hopkins/KKI faculty in 1997. Dr. Jones is currently the Administrative Laboratory Manager in the Peroxisomal Diseases Laboratory at Kennedy Krieger Institute as well as a Research Associate in Neurology at Johns Hopkins.

Dr. Jones is a member of the Society for Neuroscience, the American Association for Clinical Chemistry and the Clinical Laboratory Management Association.

Research Summary:

The primary disorder that the laboratory is concerned with is X-linked adrenoleukodystrophy (X-ALD). Elevated plasma levels of very long chain fatty acids (VLCFA) are diagnostic of X-ALD. An assay for plasma VLCFA was developed in this laboratory and is now used by many other laboratories. Use of this assay has identified more than 2500 patients with peroxisomal disorders. It is also used for prenatal diagnosis, and more than 500 pregnancies have been monitored. This laboratory is a major reference laboratory for peroxisomal disorders both nationally and internationally.

The major research objective of this laboratory is to develop an effective therapy for X-ALD and other peroxisomal disorders, a goal which is reinforced daily by contact with patients and their families. Dr. Jones has been performing the data analysis for the evaluation of Lorenzo’s Oil therapy in the treatment of X-ALD. He has also been involved in the evaluation of Lovastatin as a potential therapeutic agent in X-ALD. These studies have been conducted in cultured fibroblast cells from X-ALD patients. Studies have included the effects of lovastatin treatment and the manipulation of the cholesterol levels on the composition of very long chain fatty acids in these cells. Dr. Jones is also involved in data analysis for therapeutic trials of the peroxisomal biogenesis disorders and Down Syndrome.

Recent Publications/Presentations:

Moser HW, Raymond GV, Köhler W, Sokolowski P, Moser A, Korenke GC, Greene A, Loes DJ, Hunnemann DF, Jones RO, Lu S-E, Uziel G, Roels F. Evaulation of the preventitive effect of glyceryl trioleate-trierucate (Lorenzo’s Oil) therapy in X-linked aderenoleukodystrophy: Results of two concurrent studies. Program & Abstracts, Peroxisomal Disorders and Regulation of Genes, Ghent University, 2002.

 
Richard Jones Colleagues :
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Bryan Stark

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Elise Babbitt

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Joseph Pillion

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Roberta Mason

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Harolyn Belcher

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