Richard Hanha syndrome is caused by defective metabolism of:: (A) Lysine, (B) Arginine, (C) Tyrosine, (D) Glycine
Answer is C. Following transamination, the carbon skeleton of tyrosine is degraded to fumarate and acetoacetate. Metabolic diseases of tyrosine catabolism include tyrosinosis, Richner-Hanha syndrome, neonatal tyrosinemia, and alkaptonuria. Ref: Harper 28th edition, chapter 29.