My full name is Patric Noel Baldwin[known as Noel] Date of Birth: 20 th of January 1937 in Lismore Co-waterford Ireland Educated at the Christen

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1 My full name is Patric Noel Baldwin[known as Noel] Date of Birth: 20 th of January 1937 in Lismore Co-waterford Ireland Educated at the Christen Brothers College Lismore Retired railway civil engineering manager Councillor on the West Lindsey District Council Serves on several committees Director of the shaw trust on the board of governors for two schools. 1

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4 DR HARASH NARANG -BIOGRAPHY 1970 After gaining a PhD from Newcastle University Harash joined the Medical Research Council (MRC) as a full time Scientific Officer to work on CJD, Scapie, Alzheimer's and MS. Published various papers. 4

5 1972 Harash published first paper in Nature magazine on subject of Scrapie describing the structures named nemavirus. Subsequently published further papers on these subjects Harash decided to become a member of the Royal College of Pathologists whist working at the MRC. Prof. Field, who Harash was working with, had retired and Harash was approached by Public Health Laboratory (PHLS) to join them. It was here he got his membership of the Royal College of Pathologists and then a fellowship Harash joined PHLS with assurance that he could continue with his research in CJD, and related diseases Harash also started developing diagnostic testing relating to PHLS work and published a number of papers Prusiner's Group in the USA published a paper confirming Harash's findings of This generated interest and he was asked by a leading group of researchers headed by Prof Chandler, based in Compton (Newbury) UK to come to their laboratory and demonstrate his nemavirus particles this work was published in Pat Mertz (NY) while trying to purify nemavirus appears to have found a unique structure in affected brain samples only. These structures were called Scrapie-Associated Fibrils (SAF) and for some time these structures were considered to be the agent or part of the agent of Spongiform Encephalolopathy (SE) Prusiner found what appeared to be a unique protein, Protease resistant Protein (PrP). He called this Prion and suggested this was the agent or part of the agent. SAF described by Pat Mertz are made up if PrP. It was also established that PrP itself from the host protein by some modifications occurring only after the host has been infected Canton Gajdusek the Nobel Laureate came to Newcastle and invited Harash to join his team at the National Institute of Health (NIH) in the USA, because Harash could demonstrate a relationship between SAF, Prion and Nemavirus Eventually H decided to go to the USA to undertake this work and within only a matter of months Gadjusek made it clear in a letter to the PHLS Director that he was impressed with Harash's work and tried to persuade his employers, PHLS to extend his leave of absence. It was a co-incidence that while Harash was there, the first three cases of Growth Hormone/CJD Infection were identified and Harash was able to alert the British Health Authority. Harash, having personal experience of investigating case histories of GH victims, became aware of the differences in the clinical symptoms between these and classic CJD. Gadjusek did not receive a reply from Harash's employers PHLS relating to the absence of leave, so he offered H the status of visiting scientist to work at the NIH. This allowed their working relationship to continue. Gadjusek tried very hard to persuade Harash to become a permanent member but because of the emerging BSE crisis in the UK Harash felt obliged to stay in the UK. 5

6 1987 While working at the NIH, Harash was able to demonstrate a relationship between SAFIPrP and nemavirus. Nemavirus is a three-iayer structure, PrP/SAF is protected by another host protein SAF forms the central core while the DNA wraps around it. Various other papers were published. The significance of the work carried out by Harash at NHI was that he had developed a touch technique that could be used as a diagnostic tool for SE. Harash proposed this touch technique test to MAFF for the diagnosis of BSE. This test would have helped reduce the backlog of BSE cases awaiting confirmation and also identified affected animals being killed in local abattoirs, preventing their appearance in the human food chain. The significance of this would have been far more than to just remove the incubating animals from the human food chain, it would have given a true incident of animals affected by BSE. This process would have also identified affected farms and progeny of affected animals. The sum of all this information would have helped in controlling, and the eventual eradication of the disease Harash had a meeting with MAFF officials, consideration was given to this test and Harash was eventually asked to apply for funding. This application was subsequently turned down Ken Bell decided to fund this part of the research and Harash was able to publish a paper in the Lancet describing the high incidence of CJD in the North which would not have been found without the help of the Touch Technique. MAFF eventually agreed to supply brain samples from 10 cattle to see if this was a viable test. There was a significant delay before these samples arrived. Harash had no control over the collection of the specimens and written documents suggest that MAFF recorded samples given on different dates than those marked on the samples. Therefore, if there is a simple mix up of specimens the validity of the test is not watertight. Harash got 80% accuracy and no false positives in the diagnostic testing -excellent results under such difficult conditions MAFF refused to supply any more specimens. Harash was able to purify DNA which happened to be single strand DNA (ssdna) Harash filed a patent application for the sequence ssdna which was eventually granted for PCR detection Having worked with another human viral infection Cytomeglovirus, which is excreted in-patients urine, Harash developed a method to concentrate this virus from patients urine. Whilst working in the USA a patient was admitted with CJD and Harash managed to obtain urine samples from this patient, and was soon able to demonstrate the SAF/PrP/nemavirus in the urine. At various times Harash tried to persuade the British Government (MAFF) to provide him with urine sample. Eventually as Harash's work became known through the media, the relatives of CJD victims contacted him to carry out tests to confirm the disease. Some these patients were not even thought to have CJD by the consulting Neurologist, but all cases identified by Harash using urine test were confirmed later by post mortem

7 Full paper on ssdna was published Pruisner s group also concluded from their experiments that although they did not identify ssdna they described an identical mechanism conversion of normal PrP into SAF/PrP as previously described by Harash in As it is obvious from the mechanism described by H in 1992 ssdna initiates the formation of SAF/PrP therefore logically the prion is a by-product of the disease process and can also be used as disease markets. Their presence in any body tissue or fluid would indicate infectivity I i.e. the host is incubating or infected with disease. World in Action were able to collect brain samples from 30 cattle from an abattoir, while meat went into the human food chain and Harash found 29% to be positive Having realised the importance of this test Harash filed a 2nd patent, covering both ssdna by PCR and SAF/PrP by Western blot/elisa and EM. Recently a group of researchers led by Ruth Gabizon in Israel have demonstrated using basic technology PrP by Western blot in affected animals Harash continues his work to stem the spread of BSE, through his research, speaking at conferences in the USA, Japan and the UK and commenting in the international media. Visit his website CJD(Scrapie) MS( ) ( MRC ) 1972 (nemavirus) 1976 ( MRC ) 7

8 PHLS 1977 PHLS CJD PHLS ( ) 1981 (nemavirus) 1981 NY Scrapie-Associated Fibrils ( SAF ) SAF Spongiform Encephalopathy( SE ) 1982 PrP Prion SAF PrP PrP 1983 ( NIH ) SAF Prion Nemavirus 1985 PHLS PHLS GHCJD 8

9 3 CJD PHLS NIH BSE 1987 NIH SAFPrP Nemavirus Nemavirus PrP/SAF SAF DNA NIH SE BSE MAFF BSE BSE Gillian Turner Gillian has been the National CJD Case Co-ordinator for the CJD Support Network for the past five years. During which time she has offered support to the majority of families who have been affected by all strains of the disease. Gillian has been employed in the voluntary sector caring field for the past twenty years, some of that time working for Age Concern and the British Red Cross. Gillian has two sons, Paul a doctor, Mark an Assistant Cruise Director with Princess Cruises. Gillian's husband David is a Management Lecturer with Staffordshire University. Gillian has a post graduate diploma in marketing and an MA in Gerontology from the University of Keele. She is a member of the Department of Health CJD Incidents Panel and CJD Advice Team. 9

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