INHERITED HUMAN TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
\ɪnhˈɛɹɪtɪd hjˈuːmən tɹansmˈɪsəbə͡l spˈʌnd͡ʒɪfˌɔːm ɛnsˌɛfɐlˈɒpəθɪz], \ɪnhˈɛɹɪtɪd hjˈuːmən tɹansmˈɪsəbəl spˈʌndʒɪfˌɔːm ɛnsˌɛfɐlˈɒpəθɪz], \ɪ_n_h_ˈɛ_ɹ_ɪ_t_ɪ_d h_j_ˈuː_m_ə_n 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_z]\
Definitions of INHERITED HUMAN TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
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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
Dopamine Acetyltransferase
- An enzyme that catalyzes the of groups from acetyl-CoA to arylamines. They have wide specificity for aromatic amines, particularly serotonin, and can also catalyze acetyl transfer between arylamines without CoA. EC 2.3.1.5.
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