单词 | tyrosinosis |
释义 | tyrosinosisn. Medicine. A rare condition of unknown ætiology in which there is increased excretion of the early metabolites of tyrosine but no liver or kidney damage; also (now rare) = tyrosinaemia n. ΘΚΠ the world > health and disease > ill health > a disease > disorders of internal organs > metabolic disorders > [noun] > other metabolic disorders xanthomatosis1900 Tay-Sachs1907 ketosis1917 protein shock1917 hyperinsulinism1924 Niemann–Pick1928 tyrosinosis1932 von Gierke's disease1933 phenylketonuria1935 cystic fibrosis1938 mucoviscidosis1945 cystinosis1949 oxalosis1952 McArdle1957 orotic aciduria1959 PKU1961 sphingolipidosis1962 Reye's syndrome1965 Menkes1969 1932 G. Medes in Biochem. Jrnl. XXVI. 938 The essential feature in our patient..consists in a complete stoppage of the oxidation of tyrosine at the stage of p-hydroxyphenylpyruvic acid... Thus results an excretion not only of p-hydroxyphenylpyruvic acid and..l-p-hydroxyphenyllactic acid, but also of tyrosine and..l-3:4-dihydroxyphenylalanine. For this condition, the name tyrosinosis is proposed. 1942 Jrnl. Physiol. 101 345 In tyrosinosis l(−)-dopa was found to be excreted in the urine after oral administration of l(−)-tyrosine. 1969 New Scientist 3 July 10/1 Prevention of postnatal brain damage by dietary treatment has been reported in a number of other inborn errors of metabolism. Examples include galactosaemia, tyrosinosis, maple syrup urine disease and possibly homocystinuria. 1974 R. Passmore & J. S. Robson Compan. Med. Stud. III. xlvii. 8/1 Formerly hypertyrosinaemia or tyrosinosis was considered to be a single, exceedingly rare abnormality. 1982 T. I. Diamondstone in T. M. Devlin Textbk. Biochem. xii. 589/1 Tyrosinosis and the tyrosinemias have certain biochemical features in common. This entry has not yet been fully updated (first published 1986; most recently modified version published online June 2018). < |
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