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Decreased axon caliber and neurofilaments in hereditary motor and sensory neuropathy, type I.
Ann Neurol. 1984 Aug; 16(2):238-41.AN

Abstract

The axons of large- and intermediate-diameter myelinated fibers of sural nerves of patients with hereditary motor and sensory neuropathy, type I (HMSN-I), were previously found to be attenuated relative to their myelin spiral length. We inferred that axonal atrophy might account for secondary segmental demyelination and remyelination. To assess whether the observed axonal atrophy could be explained by a decrease in neurofilaments, we have evaluated the number of neurofilaments, microtubules, and other axon organelles in sural nerves of patients with HMSN-I. Whereas the density per square micrometer of neurofilaments or microtubules in diseased nerves was not significantly different from that in control specimens, the number of neurofilaments per axon as related to myelin spiral length was significantly less for intermediate and large myelinated fibers in HMSN-I nerves. The regression lines for the number of microtubules per axon on myelin spiral lengths were also less steep in HMSN-I, but the difference did not reach statistical significance. These results indicate that the number of neurofilaments is proportional to axon diameter but significantly below that expected considering myelin spiral length. Decreased neurofilament synthesis, assembly, or transport may underlie the axonal atrophy in HMSN-I.

Authors

No affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

6541018

Citation

Nukada, H, and P J. Dyck. "Decreased Axon Caliber and Neurofilaments in Hereditary Motor and Sensory Neuropathy, Type I." Annals of Neurology, vol. 16, no. 2, 1984, pp. 238-41.
Nukada H, Dyck PJ. Decreased axon caliber and neurofilaments in hereditary motor and sensory neuropathy, type I. Ann Neurol. 1984;16(2):238-41.
Nukada, H., & Dyck, P. J. (1984). Decreased axon caliber and neurofilaments in hereditary motor and sensory neuropathy, type I. Annals of Neurology, 16(2), 238-41.
Nukada H, Dyck PJ. Decreased Axon Caliber and Neurofilaments in Hereditary Motor and Sensory Neuropathy, Type I. Ann Neurol. 1984;16(2):238-41. PubMed PMID: 6541018.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Decreased axon caliber and neurofilaments in hereditary motor and sensory neuropathy, type I. AU - Nukada,H, AU - Dyck,P J, PY - 1984/8/1/pubmed PY - 1984/8/1/medline PY - 1984/8/1/entrez SP - 238 EP - 41 JF - Annals of neurology JO - Ann. Neurol. VL - 16 IS - 2 N2 - The axons of large- and intermediate-diameter myelinated fibers of sural nerves of patients with hereditary motor and sensory neuropathy, type I (HMSN-I), were previously found to be attenuated relative to their myelin spiral length. We inferred that axonal atrophy might account for secondary segmental demyelination and remyelination. To assess whether the observed axonal atrophy could be explained by a decrease in neurofilaments, we have evaluated the number of neurofilaments, microtubules, and other axon organelles in sural nerves of patients with HMSN-I. Whereas the density per square micrometer of neurofilaments or microtubules in diseased nerves was not significantly different from that in control specimens, the number of neurofilaments per axon as related to myelin spiral length was significantly less for intermediate and large myelinated fibers in HMSN-I nerves. The regression lines for the number of microtubules per axon on myelin spiral lengths were also less steep in HMSN-I, but the difference did not reach statistical significance. These results indicate that the number of neurofilaments is proportional to axon diameter but significantly below that expected considering myelin spiral length. Decreased neurofilament synthesis, assembly, or transport may underlie the axonal atrophy in HMSN-I. SN - 0364-5134 UR - https://www.unboundmedicine.com/medline/citation/6541018/Decreased_axon_caliber_and_neurofilaments_in_hereditary_motor_and_sensory_neuropathy_type_I_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0364-5134&date=1984&volume=16&issue=2&spage=238 DB - PRIME DP - Unbound Medicine ER -