Cargando…
Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease
Niemann-Pick Type C (NPC) is a rare genetic disorder characterized by progressive cell death in various tissues, particularly in the cerebellar Purkinje cells, with no known cure. Mouse models for human NPC have been generated and characterized histologically, behaviorally, and using longitudinal ma...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7443857/ https://www.ncbi.nlm.nih.gov/pubmed/32417449 http://dx.doi.org/10.1016/j.neuroimage.2020.116894 |
_version_ | 1783573707642372096 |
---|---|
author | Rallapalli, Harikrishna Darwin, Benjamin C. Toro-Montoya, Estefania Lerch, Jason P. Turnbull, Daniel H. |
author_facet | Rallapalli, Harikrishna Darwin, Benjamin C. Toro-Montoya, Estefania Lerch, Jason P. Turnbull, Daniel H. |
author_sort | Rallapalli, Harikrishna |
collection | PubMed |
description | Niemann-Pick Type C (NPC) is a rare genetic disorder characterized by progressive cell death in various tissues, particularly in the cerebellar Purkinje cells, with no known cure. Mouse models for human NPC have been generated and characterized histologically, behaviorally, and using longitudinal magnetic resonance imaging (MRI). Previous imaging studies revealed significant brain volume differences between mutant and wild-type animals, but stopped short of making volumetric comparisons of the cerebellar sub-regions. In this study, we present longitudinal manganese-enhanced MRI (MEMRI) data from cohorts of wild-type, heterozygote carrier, and homozygote mutant NPC mice, as well as deformation-based morphometry (DBM) driven brain volume comparisons across genotypes, including the cerebellar cortex, white matter, and nuclei. We also present the first comparisons of MEMRI signal intensities, reflecting brain and cerebellum sub-regional Mn(2+)-uptake over time and across genotypes. |
format | Online Article Text |
id | pubmed-7443857 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
record_format | MEDLINE/PubMed |
spelling | pubmed-74438572020-08-24 Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease Rallapalli, Harikrishna Darwin, Benjamin C. Toro-Montoya, Estefania Lerch, Jason P. Turnbull, Daniel H. Neuroimage Article Niemann-Pick Type C (NPC) is a rare genetic disorder characterized by progressive cell death in various tissues, particularly in the cerebellar Purkinje cells, with no known cure. Mouse models for human NPC have been generated and characterized histologically, behaviorally, and using longitudinal magnetic resonance imaging (MRI). Previous imaging studies revealed significant brain volume differences between mutant and wild-type animals, but stopped short of making volumetric comparisons of the cerebellar sub-regions. In this study, we present longitudinal manganese-enhanced MRI (MEMRI) data from cohorts of wild-type, heterozygote carrier, and homozygote mutant NPC mice, as well as deformation-based morphometry (DBM) driven brain volume comparisons across genotypes, including the cerebellar cortex, white matter, and nuclei. We also present the first comparisons of MEMRI signal intensities, reflecting brain and cerebellum sub-regional Mn(2+)-uptake over time and across genotypes. 2020-05-15 2020-08-15 /pmc/articles/PMC7443857/ /pubmed/32417449 http://dx.doi.org/10.1016/j.neuroimage.2020.116894 Text en This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Rallapalli, Harikrishna Darwin, Benjamin C. Toro-Montoya, Estefania Lerch, Jason P. Turnbull, Daniel H. Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease |
title | Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease |
title_full | Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease |
title_fullStr | Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease |
title_full_unstemmed | Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease |
title_short | Longitudinal MEMRI analysis of brain phenotypes in a mouse model of Niemann-Pick Type C disease |
title_sort | longitudinal memri analysis of brain phenotypes in a mouse model of niemann-pick type c disease |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7443857/ https://www.ncbi.nlm.nih.gov/pubmed/32417449 http://dx.doi.org/10.1016/j.neuroimage.2020.116894 |
work_keys_str_mv | AT rallapalliharikrishna longitudinalmemrianalysisofbrainphenotypesinamousemodelofniemannpicktypecdisease AT darwinbenjaminc longitudinalmemrianalysisofbrainphenotypesinamousemodelofniemannpicktypecdisease AT toromontoyaestefania longitudinalmemrianalysisofbrainphenotypesinamousemodelofniemannpicktypecdisease AT lerchjasonp longitudinalmemrianalysisofbrainphenotypesinamousemodelofniemannpicktypecdisease AT turnbulldanielh longitudinalmemrianalysisofbrainphenotypesinamousemodelofniemannpicktypecdisease |