Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes

• Main Authors: Kyoung-in Cho (Author), Dosuk Yoon (Author), Sunny Qiu (Author), Zachary Danziger (Author), Warren M. Grill (Author), William C. Wetsel (Author), Paulo A. Ferreira (Author)
• Format: Book
• Published: The Company of Biologists, 2017-05-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

The pathogenic drivers of sporadic and familial motor neuron disease (MND), such amyotrophic lateral sclerosis (ALS), are unknown. MND impairs the Ran GTPase cycle, which controls nucleocytoplasmic transport, ribostasis and proteostasis; however, cause-effect mechanisms of Ran GTPase modulators in m...