MRI detects therapeutic effects in weanling Niemann-Pick type C mice

Silvia Lope-Piedrafita; John W. Totenhagen; Chad M. Hicks; Robert P. Erickson; Theodore P. Trouard; Theodore P. Trouard

doi:https://doi.org/10.1002/jnr.21707

MRI detects therapeutic effects in weanling Niemann-Pick type C mice

Silvia Lope-Piedrafita, John W. Totenhagen, Chad M. Hicks, Robert P. Erickson, Theodore P. Trouard, Theodore P. Trouard

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

16 Citations (Scopus)

Abstract

To noninvasively evaluate the early effects of Niemann-Pick type C (NPC) disease, diffusion tensor imaging (DTI) was carried out in the brains of very young (23-day-old) mice. The diffusion of water in white matter tracts of Npc1-/- mice at this young age was already abnormal, exhibiting decreased anisotropy, as quantified by fractional anisotropy (FA), compared with their wild-type littermates, the controls. Postmortem histological staining revealed myelin de.ciencies in Npc1-/- mice, consistent with the reduction in FA measured vivo. Beneficial effects of treatment with allopregnanolone and/ or 2 hydroxypropyl-beta-cyclodextrin was also detectable at this age by FA, which correlated with increased myelination as seen by histology. This is the earliest detection of a therapeutic effect in Npc1-/- mice. © 2008 Wiley-Liss, Inc.

Original language	English
Pages (from-to)	2802-2807
Journal	Journal of Neuroscience Research
Volume	86
DOIs	https://doi.org/10.1002/jnr.21707
Publication status	Published - 8 Dec 2008

Keywords

Allopregnanolone
Beta-cyclodextrin
Brain
DTI
Dysmyelination
Mouse
Neurodegeneration
Neurosteroids

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title = "MRI detects therapeutic effects in weanling Niemann-Pick type C mice",

abstract = "To noninvasively evaluate the early effects of Niemann-Pick type C (NPC) disease, diffusion tensor imaging (DTI) was carried out in the brains of very young (23-day-old) mice. The diffusion of water in white matter tracts of Npc1-/- mice at this young age was already abnormal, exhibiting decreased anisotropy, as quantified by fractional anisotropy (FA), compared with their wild-type littermates, the controls. Postmortem histological staining revealed myelin de.ciencies in Npc1-/- mice, consistent with the reduction in FA measured vivo. Beneficial effects of treatment with allopregnanolone and/ or 2 hydroxypropyl-beta-cyclodextrin was also detectable at this age by FA, which correlated with increased myelination as seen by histology. This is the earliest detection of a therapeutic effect in Npc1-/- mice. {\textcopyright} 2008 Wiley-Liss, Inc.",

keywords = "Allopregnanolone, Beta-cyclodextrin, Brain, DTI, Dysmyelination, Mouse, Neurodegeneration, Neurosteroids",

author = "Silvia Lope-Piedrafita and Totenhagen, {John W.} and Hicks, {Chad M.} and Erickson, {Robert P.} and Trouard, {Theodore P.} and Trouard, {Theodore P.}",

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T1 - MRI detects therapeutic effects in weanling Niemann-Pick type C mice

AU - Lope-Piedrafita, Silvia

AU - Totenhagen, John W.

AU - Hicks, Chad M.

AU - Erickson, Robert P.

AU - Trouard, Theodore P.

PY - 2008/12/8

Y1 - 2008/12/8

N2 - To noninvasively evaluate the early effects of Niemann-Pick type C (NPC) disease, diffusion tensor imaging (DTI) was carried out in the brains of very young (23-day-old) mice. The diffusion of water in white matter tracts of Npc1-/- mice at this young age was already abnormal, exhibiting decreased anisotropy, as quantified by fractional anisotropy (FA), compared with their wild-type littermates, the controls. Postmortem histological staining revealed myelin de.ciencies in Npc1-/- mice, consistent with the reduction in FA measured vivo. Beneficial effects of treatment with allopregnanolone and/ or 2 hydroxypropyl-beta-cyclodextrin was also detectable at this age by FA, which correlated with increased myelination as seen by histology. This is the earliest detection of a therapeutic effect in Npc1-/- mice. © 2008 Wiley-Liss, Inc.

AB - To noninvasively evaluate the early effects of Niemann-Pick type C (NPC) disease, diffusion tensor imaging (DTI) was carried out in the brains of very young (23-day-old) mice. The diffusion of water in white matter tracts of Npc1-/- mice at this young age was already abnormal, exhibiting decreased anisotropy, as quantified by fractional anisotropy (FA), compared with their wild-type littermates, the controls. Postmortem histological staining revealed myelin de.ciencies in Npc1-/- mice, consistent with the reduction in FA measured vivo. Beneficial effects of treatment with allopregnanolone and/ or 2 hydroxypropyl-beta-cyclodextrin was also detectable at this age by FA, which correlated with increased myelination as seen by histology. This is the earliest detection of a therapeutic effect in Npc1-/- mice. © 2008 Wiley-Liss, Inc.

KW - Allopregnanolone

KW - Beta-cyclodextrin

KW - Brain

KW - DTI

KW - Dysmyelination

KW - Mouse

KW - Neurodegeneration

KW - Neurosteroids

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DO - https://doi.org/10.1002/jnr.21707

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SP - 2802

EP - 2807

JO - Journal of Neuroscience Research

JF - Journal of Neuroscience Research

SN - 0360-4012

ER -