Antibody key to treating variant CJD, scientists find

March 04, 2009

Scientists at the University of Liverpool have determined the atomic structure of the 'binding' between a brain protein and an antibody that could be key to treating patients with diseases such as variant CJD.

Variant Creutzfeldt-Jakob Disease (vCJD) is part of a family of rare progressive neurodegenerative disorders, called prion diseases, which affect both animals and humans. It is thought that those who have developed vCJD became infected through the consumption of cattle products contaminated with Bovine Spongiform Encephalopathy (BSE) - a brain disorder in cows, commonly known as Mad Cow Disease.

Prion diseases can develop when a naturally occurring brain prion protein called, PrP, comes into contact with infectious prions. This converts PrP into a form that has a different shape, and eventually leads to a build-up of protein in the brain, causing brain cells to die. It is thought that immunisation with antibodies that can 'stick' to PrP could treat and even prevent the development of the disease.

To understand the 'connection' between the antibody and the protein, scientists at Liverpool used X-ray crystallography technology to build a three-dimensional picture of the binding between an antibody called ICSM18 - designed to 'stick' effectively to prion proteins - and PrP cells.

Samar Hasnain, Professor of Molecular Biophysics at the University, explains: "To pin-point where the antibody 'sticks' to the protein we used X-ray crystallography, pioneered by Nobel Prize winner Max Perutz. Significantly we found that the point at which the protein and antibody came together was also where scientists at the Medical Research Council (MRC) Prion Unit had identified a single amino acid, which we now know has a significant impact on a patient's susceptibility to prion disease."

Scientists at the MRC Prion Unit, University College London, who collaborated on the research, have found that ICSM18 could help prevent brain cells from becoming infected as well as reverse early damage caused by the disease.

Professor John Collinge, Director of the MRC Prion Unit, added: "We have shown that ICSM18 has the highest therapeutic potential in animal and cell based studies, but we have yet to establish its impact on people who have vCJD or other prion diseases. We are currently working, however, to make human versions of the antibodies for future trials in people."

University of Liverpool

---

---

	Creutzfeldt-Jakob Disease: New Research by Michael J. Stonebrook (Editor) Creutzfeldt-Jakob Disease is a very rare and incurable degenerative neurological disorder that is ultimately fatal. It is the most common of the transmissible spongiform encephalopathies. Transmissible spongiform encephalopathy diseases (also known as prion diseases) are caused by a unique type of infectious agent called prions, an abnormally structured form of a protein found in the brain. Other prion diseases include Gerstmann-Straussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru in humans, as well as bovine spongiform encephalopathy (BSE) and scrapie in animals. This book presents the latest research in this field.
	Creutzfeldt-jakob Disease (Epidemics) by Phillip Margulies (Author) Traces the history, current issues, and future of Creutzfeldt-Jakob Disease, a rare genetic disorder that effects the brain and is part of the same group of illnesses as "mad cow disease."
	Creutzfeldt-Jakob Disease and Other Transmissible Spongiform Encephalopathies by F.O. Bastian (Author), O. Creutzfeld (Author)
	Prions: Novel Infectious Pathogens Causing Scrapie and Creutzfeldt-Jakob Disease by Stanley B. Prusiner (Author), Michael P. McKinley (Editor)
	Creutzfeldt-Jakob Disease: Managing the Risk of Transmission by Blood, Plasma, and Tissues by Marc L. Turner (Author), MB (Author), ChB (Author), PhD (Author), FRCP (Author), FRCPath (Author) Secondary transmissons of variant Creutzfeldt-Jakob disease (vCJD) by blood transfusion have raised serious concerns about disease transmissioon by blood components and tissue products. This multi-authored book byy international and national experts reprents a state of the art review of the basic science and pathogenesis of iatrogenically transmitted prion disease. Atimely resource, it examines the issues of blood and tissue infectivity and screening test development; a discussion of risk assessment and management completes the book. Topics of Interest: * Molecular poathology of prion diseases * Pathogenesis of peripherally trasmitted TSEs * Transmissible spongiform encephalopathies in animals and humans * Studies on peripheral blood infectivity and transmission * Development and...
	Gale Encyclopedia of Medicine: Creutzfeldt-Jakob disease by Larry I. Lutwick MD (Author) The article is excerpted from Gale Encyclopedia of Medicine. Consult the second edition of this authoritative, comprehensive, in-depth medical guide for information on more than 1,700 medical topics in language accessible to adult laypersons. Presented in a single alphabetical sequence, articles range in length from one or two paragraphs for minor topics, to several pages or more for major topics. Disease/disorder articles typically cover definition; description; causes and symptoms; diagnosis; treatments; prevention; and more. Test/treatment articles typically cover definition; purposes; precautions; preparation; risks; normal and abnormal results; and much more. This second edition includes more than 200 new entries, 300 updated entries, approximately 650 color images...
	Gale Encyclopedia of Nursing and Allied Health: Creutzfeldt-Jakob disease by Jr., MD, PhD, DrPH L. Fleming Fallon (Author) The article is excerpted from Gale Encyclopedia of Nursing and Allied Health. This 5-vol. set provides more than 850 entries covering topics in nursing and allied health written for students and professionals in the field. Alphabetically arranged entries cover topics in body systems and functions, conditions and common diseases, issues and theories, techniques and practices, and devices and equipment. The Encyclopedia covers all major health professions, including nursing, physical therapy, occupational therapy, respiratory therapy, medical lab technology, emergency medical technology, dental assistance, pharmacology and nutrition. In addition, the set features approximately 400 photographs and illustrations; appendices of related organizations, agencies and associations;...
	Diagnostic challenges in Creutzfeldt-Jakob disease: case report.(Case Report): An article from: Southern Medical Journal by Lal K. Tanwani (Author), Christian D. Furman (Author), Christine S. Ritchie (Author) This digital document is an article from Southern Medical Journal, published by Southern Medical Association on August 1, 2003. The length of the article is 2893 words. The page length shown above is based on a typical 300-word page. The article is delivered in HTML format and is available in your Amazon.com Digital Locker immediately after purchase. You can view it with any web browser. Citation Details Title: Diagnostic challenges in Creutzfeldt-Jakob disease: case report.(Case Report) Author: Lal K. Tanwani Publication: Southern Medical Journal (Refereed) Date: August 1, 2003 Publisher: Southern Medical Association Volume: 96 Issue: 8 Page: 832(4) Distributed by Thomson...
	The fundamentals of variant Creutzfeldt-Jakob disease.: An article from: Journal of Neuroscience Nursing by Anna Budd Fontenot (Author) This digital document is an article from Journal of Neuroscience Nursing, published by American Association of Neuroscience Nurses on December 1, 2003. The length of the article is 4192 words. The page length shown above is based on a typical 300-word page. The article is delivered in HTML format and is available in your Amazon.com Digital Locker immediately after purchase. You can view it with any web browser. Citation Details Title: The fundamentals of variant Creutzfeldt-Jakob disease. Author: Anna Budd Fontenot Publication: Journal of Neuroscience Nursing (Refereed) Date: December 1, 2003 Publisher: American Association of Neuroscience Nurses Volume: 35 Issue: 6 Page: 327(5) Distributed by Thomson...
	UK blood service warns vCJD test could cut donations.(Creutzfeldt-Jakob disease)(Brief Article): An article from: Transplant News by Transplant Communications, Inc. (Publisher) This digital document is an article from Transplant News, published by Transplant Communications, Inc. on July 27, 2001. The length of the article is 583 words. The page length shown above is based on a typical 300-word page. The article is delivered in HTML format and is available in your Amazon.com Digital Locker immediately after purchase. You can view it with any web browser. Citation Details Title: UK blood service warns vCJD test could cut donations.(Creutzfeldt-Jakob disease)(Brief Article) Publication: Transplant News (Newsletter) Date: July 27, 2001 Publisher: Transplant Communications, Inc. Volume: 11 Issue: 14 Page: NA Article Type: Brief Article Distributed by Thomson...