TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY
\tɹansmˈɪsəbə͡l spˈʌnd͡ʒɪfˌɔːm ɛnsˌɛfɐlˈɒpəθɪ], \tɹansmˈɪsəbəl spˈʌndʒɪfˌɔːm ɛnsˌɛfɐlˈɒpəθɪ], \t_ɹ_a_n_s_m_ˈɪ_s_ə_b_əl s_p_ˈʌ_n_dʒ_ɪ_f_ˌɔː_m ɛ_n_s_ˌɛ_f_ɐ_l_ˈɒ_p_ə_θ_ɪ]\
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A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA, ataxia, and a fatal outcome. Diseases in this category include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER-SCHEINKER SYNDROME; KURU; SCRAPIE; fatal familial insomnia; bovine spongiform encephalopathy (see ENCEPHALOPATHY, BOVINE SPONGIFORM); transmissible mink encephalopathy; and chronic wasting disease of mule deer and elk. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95 (23):13363-83)
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Word of the day
Procollagen Proline Dioxygenase
- mixed-function oxygenase that catalyzes hydroxylation prolyl-glycyl-containing-peptide, usually in protocollagen, hydroxyprolylglycyl-peptide. The enzyme utilizes molecular oxygen with a concomitant oxidative decarboxylation of 2-oxoglutarate to succinate. EC 1.14.11.2.
Nearby Words
- transmissible gastroenteritis virus
- transmissible gastroenteritis virus, swine
- transmissible porcine gastroenteritides
- transmissible porcine gastroenteritis
- transmissible spongiform encephalopathies
- Transmissible Spongiform Encephalopathy
- transmissible venereal tumor
- transmissible venereal tumors
- transmission
- transmission channel
- transmission computed tomographies