TY - JOUR
T1 - Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A
AU - Koike, Haruki
AU - Iijima, Masahiro
AU - Mori, Keiko
AU - Yamamoto, Masahiko
AU - Hattori, Naoki
AU - Katsuno, Masahisa
AU - Tanaka, Fumiaki
AU - Watanabe, Hirohisa
AU - Doyu, Manabu
AU - Yoshikawa, Hiroo
AU - Sobue, Gen
PY - 2007/11
Y1 - 2007/11
N2 - Electron microscopic examination was performed to compare morphologic changes of nonmyelinating Schwann cells and unmyelinated axons in patients with Charcot-Marie-Tooth disease type 1A (CMT1A) with peripheral myelin protein 22 duplication (n = 27) and normal control individuals (n = 14). Complete transverse sural nerve cross-sections were obtained in 16 patients and the total number of axons and Schwann cells in each cross-section was estimated. In patients with CMT1A, the number of myelinated axons was significantly decreased, whereas unmyelinated axons were well-preserved and did not show any marked changes. The numbers of nuclei, subunits, and profiles of nonmyelinating Schwann cells were all increased significantly in patients with CMT1A, whereas the numbers of axons per unmyelinated axon-containing subunit were significantly decreased. Schwann cell subunits consisted of layers of flattened cytoplasmic profiles wrapped around unmyelinated axons in the patient with CMT1A. The numbers of nonmyelinating Schwann cell profiles were increased and the numbers of axons per unmyelinated axon-containing subunit were reduced even in young patients with well-preserved myelinated fibers. In conclusion, there is marked alteration of the population and morphology of nonmyelinating Schwann cells, and axon-Schwann cell interactions seem to be regulated differently between myelinated and unmyelinated fibers in CMT1A.
AB - Electron microscopic examination was performed to compare morphologic changes of nonmyelinating Schwann cells and unmyelinated axons in patients with Charcot-Marie-Tooth disease type 1A (CMT1A) with peripheral myelin protein 22 duplication (n = 27) and normal control individuals (n = 14). Complete transverse sural nerve cross-sections were obtained in 16 patients and the total number of axons and Schwann cells in each cross-section was estimated. In patients with CMT1A, the number of myelinated axons was significantly decreased, whereas unmyelinated axons were well-preserved and did not show any marked changes. The numbers of nuclei, subunits, and profiles of nonmyelinating Schwann cells were all increased significantly in patients with CMT1A, whereas the numbers of axons per unmyelinated axon-containing subunit were significantly decreased. Schwann cell subunits consisted of layers of flattened cytoplasmic profiles wrapped around unmyelinated axons in the patient with CMT1A. The numbers of nonmyelinating Schwann cell profiles were increased and the numbers of axons per unmyelinated axon-containing subunit were reduced even in young patients with well-preserved myelinated fibers. In conclusion, there is marked alteration of the population and morphology of nonmyelinating Schwann cells, and axon-Schwann cell interactions seem to be regulated differently between myelinated and unmyelinated fibers in CMT1A.
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U2 - 10.1097/NEN.0b013e3181598294
DO - 10.1097/NEN.0b013e3181598294
M3 - Article
C2 - 17984684
AN - SCOPUS:35848962730
SN - 0022-3069
VL - 66
SP - 1027
EP - 1036
JO - Journal of Neuropathology and Experimental Neurology
JF - Journal of Neuropathology and Experimental Neurology
IS - 11
ER -