PMID: 41876Dec 1, 1979

Hepatic enzymes of tyrosine metabolism in tyrosinemia II

The Journal of Investigative Dermatology
L A GoldsmithC R Roe


A middle-aged adult male with a mild form of tyrosinemia II (Richner-Hanhart syndrome) is described. Treatment with a low-tyrosine diet caused a fall in plasma tyrosine and clearing of the hyperkeratosis of the soles. Liver biopsy of this patient revealed low but measurable levels of cytoplasmic tyrosine aminotransferase and elevated levels of the mitochondrial tyrosine-metabolizing enzyme aspartate aminotransferase. It is hypothesized that these enzymes have been induced in sufficient amounts to account for the mild clinical course.


Jul 1, 1983·Pediatric Dermatology·L A Goldsmith
Jun 10, 2004·The Journal of Dermatology·L A Goldsmith
May 11, 2006·Journal of the European Academy of Dermatology and Venereology : JEADV·M ValikhaniS Toosi
Nov 1, 1987·Clinical and Experimental Dermatology·N G FraserJ L McPhie
Jan 1, 1982·Journal of Inherited Metabolic Disease·K KidaY Miki
Jun 1, 1985·International Journal of Dermatology·L A Goldsmith
Jul 1, 1994·The British Journal of Dermatology·G P LuckerP M Steijlen

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