The enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT) catalyzes the reutilization of hypoxanthine and guanine to the purine nucleotides IMP and GMP, respectively. HPRT deficiency is an X-linked disorder characterized by uric acid overproduction and variable neurologic impairment. The complete deficiency of HPRT is diagnostic of Lesch-Nyhan syndrome manifested by choreoathetosis, spas...

Subjects with the Lesch-Nyhan syndrome (hypoxanthine-guanine phosphoribosyltransferase deficiency with self-mutilation) exhibit an apparently unique pattern of adrenergic dysfunction characterized by elevated plasma dopamine beta-hydroxylase activity and an absence of pressor response to acute sympathetic stimulation. Patients with a partial deficiency of hypoxanthine-guanine phosphoribosyltran...

Lesch-Nyhan syndrome (LNS) is a rare inherited disorder caused by a deficiency of the enzyme hypoxanthine-guanine phosphoribosyl transferase-1. Few reports on behavioral aspects especially self-injurious behavior in LNS patients are available. We report a case of LNS in an 8-year-old male child, who presented with characteristic self-injurious behavior.

We prove that the index formula for b-elliptic cone differential operators given by Lesch in [6] holds verbatim for operators whose coefficients are not necessarily independent of the normal variable near the boundary. We also show that, for index purposes, the operators can always be considered on weighted Sobolev spaces.

Andreas Lesch, Po-Chung Chen, Folkert Roelfs, Carsten Dosche, Dmitry Momotenko, Fernando Cortés-Salazar, Hubert H. Girault and Gunther Wittstock † Carl von Ossietzky University of Oldenburg, School of Mathematics and Natural Sciences, Center of Interface Science, Department of Chemistry, D-26111 Oldenburg, Germany ‡ Ecole Polytechnique Fédérale de Lausanne, Laboratoire d’Electrochimie Physique ...

A new nor-cardenolide, named toxicarioside H (1), was isolated from the latex of Antiaris toxicaria (Pers.) Lesch (Moraceae). Its structure was elucidated on the basis of HRFAB-MS and spectroscopic techniques (IR, UV, 1D and 2D NMR). Compound 1 showed significant cytotoxicity against K562, SGC-7901, SMMC-7721, and HeLa cell lines in vitro by MTT method.