Phenotype Image Detail

Image

Caption

Neuronal loss and cytopathology of the spinal motor neurons in Psmc4^tm1.1Ryot/Psmc4^tm1.2Ryot Tg(SLC18A3-cre)KMisa/0 (Rpt3-CKO) mice. A, Choline acetyltransferase (ChAT) immunohistochemistry of spinal anterior horn cells from mutant mice (d-f) and control mice (a-c) at 6, 12, and 40 weeks of age. The number of motor neurons, indicated by ChAT-positive immunoreactivity, in mutant mice is significantly lower at 12 and 40 weeks of age than that of control mice (g) (n=3, p<0.01). NS indicates not significant. B, H&E staining of anterior horns from control (a-c) and mutant mice (d-f). Mutant mice (12 weeks) have abnormal motor neurons with eosinophilic substances in the cytosol (arrows). C, spinal motor neurons from mutant mice show chromatolysis and basophilic inclusions, resembling those in Amyotrophic Lateral Sclerosis (ALS) patients. a and c, Nissl staining; b and d, H&E staining. Scale bars, 30 um (A), 20 um (B), and 10 um (C).

Copyright

This image is from Tashiro Y, J Biol Chem 2012 Dec 14;287(51):42984-94 and is displayed with the permission of the American Society for Biochemistry and Molecular Biology who owns the Copyright. Full text from JBC. J:193770

Associated Alleles

Symbol	Name
Psmc4^tm1.1Ryot	proteasome (prosome, macropain) 26S subunit, ATPase, 4; targeted mutation 1.1, Ryosuke Takahashi
Psmc4^tm1.2Ryot	proteasome (prosome, macropain) 26S subunit, ATPase, 4; targeted mutation 1.2, Ryosuke Takahashi
Tg(SLC18A3-cre)KMisa	transgene insertion K, Hidemi Misawa

Associated Genotypes

Allelic Composition	Genetic Background
Psmc4^tm1.1Ryot/Psmc4^tm1.2Ryot Tg(SLC18A3-cre)KMisa/0	involves: C57BL/6 * C57BL/6N