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ENZYMATIC STUDIES IN A CASE OF HEREDITARY TYROSINEMIA WITH HEPATOMA
Author(s) -
GENTZ J.,
HEINRICH J.,
LINDBLAD B.,
LINDSTEDT S.,
ZETTERSTRÖM R.
Publication year - 1969
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1969.tb04736.x
Subject(s) - tyrosinemia , enzyme , enzyme assay , medicine , liver tissue , urine , endocrinology , tyrosine aminotransferase , tyrosine , biochemistry , chemistry , enzyme inducer
Summary The activity of δ‐aminolevulinic acid synthetase has been measured in liver and hepatoma tissue from a case of hereditary tyrosinemia with an increased excretion of δ‐aminolevulinic acid in the urine. The enzyme activity in the hepatoma tissue was seven times higher than in the surrounding liver, where the activity was not increased above the reported normal range. The activity of p ‐hydroxyphe‐nylpyruvate hydroxylase (EC 1.14.2.2.) was about 25 % of the normal in the liver tissue but not measurable in the hepatoma. Tyrosine aminotransferase (EC 2.6.1.5.) activity was normal in the liver tissue but was not detectable in the hepatoma, whereas the activity of phenylpy‐ruvate keto‐enol isomerase (EC 5.3.2.1.) was the same in liver and hepatoma tissue.