Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation

Akira Kondo; Peter M. Hoogerbrugge; Kinuko Suzuki; Ben J.H.M. Poorthuis; Dirk W. Van Bekkum; Kunihiko Suzuki

doi:10.1016/0006-8993(88)91220-6

Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation

Akira Kondo, Peter M. Hoogerbrugge, Kinuko Suzuki, Ben J.H.M. Poorthuis, Dirk W. Van Bekkum, Kunihiko Suzuki

Research output: Contribution to journal › Article › peer-review

3 Citations (Scopus)

Abstract

The peripheral nerve of the homozygous twitcher mouse (twi/twi), a murine model of globoid cell leukodystrophy (GLD), was examined following bone marrow transplantation (BMT). The light and electron microscopic studies revealed markedly increased numbers of remyelinated fibers and almost complete disappearance of the typical inclusion-laden macrophages in the trigeminal and sciatic nerves of the twi/twi which survived beyond 100 days of age. The pattern of remyelination appeared to be normal. GLD inclusions were still observed in the cytoplasm of some of the remyelinating Schwann cells and demyelinated fibers were still present in 108-day-old twitcher although no features of active demyelinating processes were observed. Thus, basic metabolic abnormality is still present despite clinical improvement in the twi/twi mouse following BMT.

Original language	English
Pages (from-to)	178-183
Number of pages	6
Journal	Brain Research
Volume	460
Issue number	1
DOIs	https://doi.org/10.1016/0006-8993(88)91220-6
Publication status	Published - 13 Sept 1988
Externally published	Yes

Keywords

Bone marrow transplantation
Globoid cell leukodystrophy
Peripheral nerve
Remyelination
Twitcher mouse

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10.1016/0006-8993(88)91220-6

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title = "Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation",

abstract = "The peripheral nerve of the homozygous twitcher mouse (twi/twi), a murine model of globoid cell leukodystrophy (GLD), was examined following bone marrow transplantation (BMT). The light and electron microscopic studies revealed markedly increased numbers of remyelinated fibers and almost complete disappearance of the typical inclusion-laden macrophages in the trigeminal and sciatic nerves of the twi/twi which survived beyond 100 days of age. The pattern of remyelination appeared to be normal. GLD inclusions were still observed in the cytoplasm of some of the remyelinating Schwann cells and demyelinated fibers were still present in 108-day-old twitcher although no features of active demyelinating processes were observed. Thus, basic metabolic abnormality is still present despite clinical improvement in the twi/twi mouse following BMT.",

keywords = "Bone marrow transplantation, Globoid cell leukodystrophy, Peripheral nerve, Remyelination, Twitcher mouse",

author = "Akira Kondo and Hoogerbrugge, {Peter M.} and Kinuko Suzuki and Poorthuis, {Ben J.H.M.} and {Van Bekkum}, {Dirk W.} and Kunihiko Suzuki",

note = "Funding Information: This researchw as supportedin part by NS-24453, NS-24928a nd HD-03110f rom the NationalI nstitute of Health, U.S.A. and Grant 28-1021f rom the 'Pre-ventie Fonds', The NetherlandsT.h e authorst hank Mrs. Clarita Langamanfo r her excellentte chnicaal s-sistancea nd Mrs. Deborah H. Sears for manuscript preparations.",

year = "1988",

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doi = "10.1016/0006-8993(88)91220-6",

language = "English",

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pages = "178--183",

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T1 - Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation

AU - Kondo, Akira

AU - Hoogerbrugge, Peter M.

AU - Suzuki, Kinuko

AU - Poorthuis, Ben J.H.M.

AU - Van Bekkum, Dirk W.

AU - Suzuki, Kunihiko

N1 - Funding Information: This researchw as supportedin part by NS-24453, NS-24928a nd HD-03110f rom the NationalI nstitute of Health, U.S.A. and Grant 28-1021f rom the 'Pre-ventie Fonds', The NetherlandsT.h e authorst hank Mrs. Clarita Langamanfo r her excellentte chnicaal s-sistancea nd Mrs. Deborah H. Sears for manuscript preparations.

PY - 1988/9/13

Y1 - 1988/9/13

N2 - The peripheral nerve of the homozygous twitcher mouse (twi/twi), a murine model of globoid cell leukodystrophy (GLD), was examined following bone marrow transplantation (BMT). The light and electron microscopic studies revealed markedly increased numbers of remyelinated fibers and almost complete disappearance of the typical inclusion-laden macrophages in the trigeminal and sciatic nerves of the twi/twi which survived beyond 100 days of age. The pattern of remyelination appeared to be normal. GLD inclusions were still observed in the cytoplasm of some of the remyelinating Schwann cells and demyelinated fibers were still present in 108-day-old twitcher although no features of active demyelinating processes were observed. Thus, basic metabolic abnormality is still present despite clinical improvement in the twi/twi mouse following BMT.

AB - The peripheral nerve of the homozygous twitcher mouse (twi/twi), a murine model of globoid cell leukodystrophy (GLD), was examined following bone marrow transplantation (BMT). The light and electron microscopic studies revealed markedly increased numbers of remyelinated fibers and almost complete disappearance of the typical inclusion-laden macrophages in the trigeminal and sciatic nerves of the twi/twi which survived beyond 100 days of age. The pattern of remyelination appeared to be normal. GLD inclusions were still observed in the cytoplasm of some of the remyelinating Schwann cells and demyelinated fibers were still present in 108-day-old twitcher although no features of active demyelinating processes were observed. Thus, basic metabolic abnormality is still present despite clinical improvement in the twi/twi mouse following BMT.

KW - Bone marrow transplantation

KW - Globoid cell leukodystrophy

KW - Peripheral nerve

KW - Remyelination

KW - Twitcher mouse

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VL - 460

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JO - Brain Research

JF - Brain Research

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Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation

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Keywords

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