<prompt> The enzyme deficient in Lesch-Nyhan syndrome isTranscarboxylaseHGPGTGlutaminase  <response>: DISEASE DEFICIENT ENZYME 1. Acute intermittent porphyria Uroporphyinogen -I synthetase 2. Albinism Tyosinase 3. Alkaptonuria Homogentisic acid oxidase 4. AP ase deficiency APase 5. Cystic fibrosis CFTR gene mutations 6. Fructose intolerance Fructose-1-P-aldolase 7. Galactosemia galactose -1-- phosphate uridyl transferase 8. Gauchers disease Beta glucosidase 9. GPD deficiency G6PD 10. Gout Increased uric acid pool 11. Isovaleric aciduria Isovaleryl CoA dehydrogenase 12. Lactic acidosis, PD deficiency Pyruvate dehydrogenase 13. Lactose intolerance Lactase 14. LESCH- NYHAN SYNDROME HGPase 15. Orotic aciduria Orotidine-5-P decaroxylase 16. Niemann-Pick disease Sphingomyelinase 17. Tays Sachs disease Hexosamindase A 18. von Gierkes disease Glucose-6-phosphate 19. Wilsons disease Copper transpo protein REF : DM VASUDEVAN TEXTBOOK ;7th EDITION , Page no :430 