Abstract
The fate of Schwann cells in Charcot-Marie-Tooth (CMT) neuropathies was addressed in this study of nerve biopsies from patients with proven PMP22 duplications and deletions. In frozen sections, apoptotic nuclei were detected using the TUNEL method. In adjacent sections, anti-neurofilament 68kD antibody was used as an axonal marker, while the antibodies to NKH-1 and low-affinity nerve growth factor receptor P75NTR were used as Schwann cell markers. In addition, plastic sections were used to determine the densities of myelinated fibers and Schwann cell nuclei. In all biopsies from CMT1A, TUNEL-positive nuclei appeared in clusters. In adjacent sections, areas of TUNEL-positive nuclei matched with areas devoid of neurofilaments and NKH-1-positive Schwann cell silhouettes, suggesting that the apoptotic nuclei belonged to nonmyelinating Schwann cells. In addition, quantitative studies on plastic-embedded sections showed a significantly reduced number of total Schwann cells compared with controls, strongly favoring a loss of Schwann cell by apoptosis. In HNPP, the number of total Schwann cells was increased and a significant Schwann cell apoptosis was observed in only 2 patients. Examination of plastic sections and teased nerve prepar...Continue Reading
Citations
Dec 10, 1999·Journal of Neurobiology·C L DelaneyE L Feldman
Dec 12, 2012·Molecular Neurobiology·Jun LiJiasong Guo
Nov 24, 1999·The American Journal of Medicine·P SaikumarM A Venkatachalam
Apr 18, 2002·Journal of Neuroimmunology·Giancarlo ContiGuglielmo Scarlato
Oct 3, 1999·Neuroscience Letters·C C Ferri, M A Bisby
Jan 11, 2002·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·J DackovićS Apostolski
Dec 10, 1999·Annals of the New York Academy of Sciences·U Suter, K A Nave
Dec 10, 1999·Annals of the New York Academy of Sciences·G ContiG Scarlato
Oct 29, 2013·Molecular Therapy : the Journal of the American Society of Gene Therapy·Zarife SahenkJerry R Mendell
Jul 17, 2008·Cellular Immunology·Alejandro LealNeil H Riordan
Oct 20, 2006·Bone·Mauro KramperaBruno Bonetti
Dec 8, 2006·Developmental Biology·Ai-Sun TsengMichael Levin
Nov 12, 2014·Scientific Reports·Taichi HaraKen Sato
Oct 1, 1999·Annals of the New York Academy of Sciences·Ueli Suter, Klaus-Armin Nave
Oct 1, 1999·Annals of the New York Academy of Sciences·G ContiG Scarlato
Dec 11, 1999·Current Opinion in Neurology·A Schenone, G L Mancardi
Jun 11, 2003·Journal of the Peripheral Nervous System : JPNS·Zarife SahenkHaikady N Najagara
Nov 12, 2017·Scientific Reports·Yi-Chu LiaoYi-Chung Lee
Mar 29, 2001·Journal of Neuropathology and Experimental Neurology·C L DelaneyE L Feldman
Oct 17, 2003·Clinics in Sports Medicine·Jun YuanGeorge A C Murrell