Lesch-Nyhan syndrome is due to the deficiency of which enzyme?: (A) Glucose 6- phosphatase, (B) Glutathione reductase, (C) PRPP glutamyl-amidotransferase, (D) Hypoxanthine-guanine phosphoribosyl transferase
Answer is D. (D) Hypoxanthine-guanine phosphoribosyl transferase # LESCH-NYHAN SYNDROMEis a sex-linked metabolic disorder since the structural gene for HGPRT is located on the X-chromosome.> It affects only the males and is characterized by excessive uric acid production (often gouty arthritis), and neurological abnormalities such as mental retardation, aggressive behaviour, learning disability etc.> The patients of this disorder 'nave an irresistible urge to bite their fingers and lips, often causing self-mutilation.