Intranuclear inclusions in subtypes of striatal neurons in Huntington's disease transgenic mice

CM Kosinski, JH Cha, AB Young, L Mangiarini… - …, 1999 - journals.lww.com
R6/2 transgenic mice express exon 1 of an abnormal human Huntington's disease (HD)
gene and develop a neurological phenotype similar to HD. These mice develop
ubiquitinated neuronal intranuclear inclusions (NII) which might play a central role in the
pathophysiology of HD. We studied the distribution of NII in subpopulations of striatal
neurons in 12-week-old R6/2 transgenic mice using fluorescent double label
immunohistochemistry. We observed that most of the Calbindin-D28K positive projection …