Biopolym. Cell. 1991; 7(3):24-30.
Молекулярних дефект виведення міді через жовч у хворих на гепатолентикулярну дегенерацію
1Пучкова Л. В., 1Вербина І. О., 1Денежкіна В. В., 1Гайцхокі В. С., 1Нейфах С. О.
  1. Інститут експериментальної медицини АМН СРСР
    Ленінград, СРСР

Abstract

Методами імуноелектрофорезу проведено скринінг зразків сироватки крові гомо- та гетерозіготних носіїв мутації Вільсона. Показано, що поряд з нормальним церулоплазміном (ЦП) у сироватці носіїв вільсонівської мутації присутній специфічний для цієї мутації ЦП-подібний білок (вільсонівський). У здорових людей ідентичний вільсонівському ЦП-подібний білок бере участь у виведенні міді з організму через жовч. Обговорюються механізми дефекту цього процесу у хворих хворобою Вільсона.

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