BSE Linked to CJD(evidence)

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pick yourself up a wall street journal, they interviewed Terry. got the full textof the article last week but was embargoed and i held out until i couldhold out no more;-)Medical Sciences Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease Cristina Casalone *{dagger} , Gianluigi Zanusso {dagger} {ddagger} , Pierluigi Acutis *, Sergio Ferrari {ddagger} , Lorenzo Capucci § , Fabrizio Tagliavini ¶, Salvatore Monaco {ddagger} ||, and Maria Caramelli * *Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; {ddagger} Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico

G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; § Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003) Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular,

and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis

showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease. ------ {dagger} C.C. and G.Z. contributed equally to this work. ||To whom correspondence should be addressed. E-mail: salvatore.monaco <salvatore.monaco. www.pnas.org/cgi/doi/10.1073/pnas.0305777101 http://www.pnas.org/cgi/content/abstract/0305777101v1 TSS The Wall Street Journal Health Tuesday, February 17, 2004 New Mad-Cow Variant Is Suspected Italian Study Raises Concern Of Disease's Broader Threat, Backing Call for Wider Tests By ANTONIO REGALADO Staff Reporter of THE WALL STREET JOURNAL Italian scientists have discovered that cattle may harbor a new form of mad-cow disease. The research by scientists at the University of Verona, in Italy, builds on earlier work that raised the possibility of unusual cases of the fatal brain-wasting disease, formally known as bovine spongiform encephalopathy. The findings pose new questions

about the risk of consuming tainted meat. "If you eat cattle with this new strain, you could get a disease in humans," said Salvatore Monaco, a biologist who made the finding with colleague Gianluigi Zanusso. Dr. Monaco said he found that the brains of certain infected animals looked similar to human brains destroyed by Creutzfeldt-Jakob disease, a neurological disorder that is believed to arise spontaneously and hadn't previously been linked to tainted meat. The human form of mad-cow disease is called variant Creutzfeldt-Jakob disease, which manifests itself differently. Now the question is whether the new form of BSE could be responsible for other Creutzfeldt-Jakob cases. About 150 cases of variant Creutzfeldt-Jakob disease -- nearly all of them in Britain -- have been linked to eating infected cattle. But the majority of cases of Creutzfeldt-Jakob, which strikes about one in a million people each year, are

termed "sporadic" because they don't have any known cause. Consumer groups are likely to leap at the Italian finding, published in the Proceedings of the National Academy of Science. Some activists have long contended that humans are contracting mad-cow disease in the U.S., contrary to what public-health officials say. "I have been waiting for this," said Terry S. Singeltary, a Bacliff, Texas, resident whose mother died in 1997 of Creutzfeldt-Jakob disease. Mr. Singeltary and others suspect that some cases of sporadic Creutzfeldt-Jakob are caused by something in the environment, perhaps contaminated meat. BSE is believed to be caused by misshapen proteins called prions that can be spread by consumption of contaminated tissue. The U.S. found its first case of the cattle disease in December, after a Holstein in Washington state tested positive for the condition. The Italian findings give some support to the

theory that BSE could be responsible for some cases of Creutzfeldt-Jakob disease, Dr. Monaco said. The Italian group studied the brains of eight cattle that had tested positive for BSE using standard tests. Six of the animals' brains showed the usual signs, but the brains of two animals showed unusual deposits of prions. The distinctive patterns of deposits may indicate a new strain of mad-cow disease, which the Italian scientists have dubbed BASE, for bovine amyloidotic spongiform encephalopathy. Linda Detweiler, a former senior veterinarian for the U.S. Department of Agriculture, said the finding isn't a complete surprise. Studies in the U.K., France and Japan have hinted that some cattle may have been suffering from atypical cases of mad-cow disease. A related disease in sheep, called scrapie, is known to come in many different strains. Dr. Detweiler said more research will be needed to confirm whether there

is in fact a new strain of BSE. One possibility is that the disease simply looks different in the specific cattle breeds in the Italian study. "I think there are still a lot of questions," Dr. Detweiler said. Stanley Prusiner, a leading researcher at the University of California, San Francisco, who is the discoverer of the prion and who reviewed the Italian report, has said the possibility of unusual BSE cases is an argument in favor of more comprehensive testing of cattle destined for human consumption. Currently, the Agriculture Department tests only a tiny fraction of cattle for mad-cow disease. Its policy is to target animals that aren't able to walk, known as downer animals, based on the theory that those are most likely to have BSE. But Dr. Monaco said his findings argue strongly for more testing in the U.S. The two animals found to have the new strain were 11 and 15 years old, "weren't acting abnormal,

and were apparently free of neurological problems," Dr. Monaco said. In Europe, all cattle slaughtered that are more than 30 months of age are tested for BSE. In Japan, all cattle turned into food are tested for the disease. Scientists first discovered that BSE could affect humans in 1996, when doctors in Britain -- where mad-cow disease was ravaging herds -- found young people succumbing to Creutzfeldt-Jakob disease, which mainly strikes older people. Further research showed the disease, which remains extremely rare, was being caused by the same strain of prion as BSE -- the pattern of damage to the brain was similar in humans and cattle, as were the molecular signatures of the prions. The existence of a second strain of BSE could mean some people are contracting it, but that the cases aren't being diagnosed properly. Write to Antonio Regalado at antonio.regalado <antonio.regalado Updated February 17, 2004 http://online.wsj.com/ to be continued.......TSS

 

 

 

 

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