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

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

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...

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Bibliographic Details
Main Authors: Kyoung-in Cho, Dosuk Yoon, Sunny Qiu, Zachary Danziger, Warren M. Grill, William C. Wetsel, Paulo A. Ferreira
Format: Article
Language:English
Published: The Company of Biologists 2017-05-01
Series:Disease Models & Mechanisms
Subjects:
Ran-binding protein 2
Chemokine signaling
Transcriptomics
Proteostasis
Motoneuron
Mouse gene knock-out
Nucleocytoplasmic transport
Metalloproteinase
Amyotrophic lateral sclerosis
Online Access:http://dmm.biologists.org/content/10/5/559

Internet

http://dmm.biologists.org/content/10/5/559

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