PMID: 7528964Jan 1, 1994Paper

Spacio-temporal progression of demyelination in twitcher mouse: with clinico-pathological correlation

Acta Neuropathologica
M Taniike, K Suzuki

Abstract

The twitcher (twi/twi) is an authentic murine model of human globoid cell leukodystrophy (GLD), caused by a deficiency of galactosylceramidase. Similar to human GLD, the twitcher shows progressive deterioration of neurological function and its neuropathology is characterized by a collection of periodic acid-Schift stain (PAS)-positive macrophages in the areas of demyelination. However, there are some differences in the clinico-pathological aspects between human and murine GLD. We investigated the spacio-temporal progression of neuropathology in the twitcher from postnatal day (PND) 10 to 45. No clinical symptoms or neuropathological changes were apparent in twi/twi until PND 15. Generally, infiltration of macrophages, concomitant with myelin degeneration, was recognized in the cerebellar white matter and the brain stem after PND 20, then in cerebral white matter after PND 25, and in cerebral and cerebellar gray matter after PND 30. The demyelination was very severe in the radix of the 8th and the 5th cranial nerves. The neurological symptoms such as tremor, spasticity and cranial nerve dysfunction were well correlated with the progression of pathological changes. Demyelination progressed in an orderly fashion such that myelin d...Continue Reading

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Citations

Oct 8, 2011·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Ian D DuncanSu-Chun Zhang
Mar 1, 1997·Journal of Neuroimmunology·S M LeVine, D C Brown
Mar 10, 2010·Journal of Lipid Research·You-Hai XuGregory A Grabowski
Mar 11, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Yoichi KondoIan D Duncan
Sep 21, 2011·ASN Neuro·Benjamin SmithErnesto R Bongarzone
Apr 14, 2016·Experimental Neurology·Ian D Duncan, Abigail B Radcliff
Feb 14, 2012·Neurobiology of Disease·Ludovico Cantuti-CastelvetriErnesto R Bongarzone
May 10, 2015·Experimental Neurology·Frank MatthesUlrich Matzner
Jul 23, 1999·Experimental Neurology·T V Pedchenko, S M LeVine
Apr 27, 2013·Human Molecular Genetics·Gregory B PotterMagdalena A Petryniak
Sep 28, 2018·The International Journal of Neuroscience·Hamdino AttiaAhmed Abdellatif
Aug 5, 2004·Journal of Neuropathology and Experimental Neurology·Takashi YagiKinuko Suzuki
Jul 20, 2000·Journal of Neuropathology and Experimental Neurology·Y P WuK Suzuki
Nov 15, 2001·Journal of Neuropathology and Experimental Neurology·Y P WuK Suzuki
Aug 26, 2016·Journal of Neuroscience Research·Yoichi Kondo, Ian D Duncan
Sep 18, 2016·Journal of Neuroscience Research·Christina R Mikulka, Mark S Sands
Jul 6, 2005·Molecular Therapy : the Journal of the American Society of Gene Therapy·Darshong LinMark S Sands
Jun 27, 2021·Human Molecular Genetics·María B Cachón-GonzálezTimothy M Cox

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