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Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A.
J Neuropathol Exp Neurol. 2007 Nov; 66(11):1027-36.JN

Abstract

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.

Authors+Show Affiliations

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

17984684

Citation

Koike, Haruki, et al. "Nonmyelinating Schwann Cell Involvement With Well-preserved Unmyelinated Axons in Charcot-Marie-Tooth Disease Type 1A." Journal of Neuropathology and Experimental Neurology, vol. 66, no. 11, 2007, pp. 1027-36.
Koike H, Iijima M, Mori K, et al. Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A. J Neuropathol Exp Neurol. 2007;66(11):1027-36.
Koike, H., Iijima, M., Mori, K., Yamamoto, M., Hattori, N., Katsuno, M., Tanaka, F., Watanabe, H., Doyu, M., Yoshikawa, H., & Sobue, G. (2007). Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A. Journal of Neuropathology and Experimental Neurology, 66(11), 1027-36.
Koike H, et al. Nonmyelinating Schwann Cell Involvement With Well-preserved Unmyelinated Axons in Charcot-Marie-Tooth Disease Type 1A. J Neuropathol Exp Neurol. 2007;66(11):1027-36. PubMed PMID: 17984684.
* Article titles in AMA citation format should be in sentence-case
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 - 2007/11/7/pubmed PY - 2007/12/14/medline PY - 2007/11/7/entrez SP - 1027 EP - 36 JF - Journal of neuropathology and experimental neurology JO - J. Neuropathol. Exp. Neurol. VL - 66 IS - 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. SN - 0022-3069 UR - https://www.unboundmedicine.com/medline/citation/17984684/Nonmyelinating_Schwann_cell_involvement_with_well_preserved_unmyelinated_axons_in_Charcot_Marie_Tooth_disease_type_1A_ L2 - https://academic.oup.com/jnen/article-lookup/doi/10.1097/NEN.0b013e3181598294 DB - PRIME DP - Unbound Medicine ER -