JUVENILE HUNTINGTON DISEASE
\d͡ʒˈuːvənˌa͡ɪl hˈʌntɪŋtən dɪzˈiːz], \dʒˈuːvənˌaɪl hˈʌntɪŋtən dɪzˈiːz], \dʒ_ˈuː_v_ə_n_ˌaɪ_l h_ˈʌ_n_t_ɪ_ŋ_t_ə_n d_ɪ_z_ˈiː_z]\
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A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse CHOREA involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
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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.
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