@article {1503169,
	title = {ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair},
	journal = {Nat Neurosci},
	volume = {22},
	number = {2},
	year = {2019},
	month = {2019 02},
	pages = {167-179},
	abstract = {The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its gene cause familial ALS have nominated altered RNA metabolism as a disease mechanism. However, the RNAs regulated by TDP-43 in motor neurons and their connection to neuropathy remain to be identified. Here we report transcripts whose abundances in human motor neurons are sensitive to TDP-43 depletion. Notably, expression of STMN2, which encodes a microtubule regulator, declined after TDP-43 knockdown and TDP-43 mislocalization as well as in patient-specific motor neurons and postmortem patient spinal cord. STMN2 loss upon reduced TDP-43 function was due to altered splicing, which is functionally important, as we show STMN2 is necessary for normal axonal outgrowth and regeneration. Notably, post-translational stabilization of STMN2 rescued neurite outgrowth and axon regeneration deficits induced by TDP-43 depletion. We propose that restoring STMN2 expression warrants examination as a therapeutic strategy for ALS.},
	keywords = {Amyotrophic Lateral Sclerosis, Axons, Cell Line, DNA-Binding Proteins, Down-Regulation, Female, Humans, Induced Pluripotent Stem Cells, Male, Membrane Proteins, Motor Neurons, Spinal Cord},
	issn = {1546-1726},
	doi = {10.1038/s41593-018-0300-4},
	author = {Klim, Joseph R and Williams, Luis A and Limone, Francesco and Guerra San Juan, Irune and Davis-Dusenbery, Brandi N and Mordes, Daniel A and Burberry, Aaron and Steinbaugh, Michael J and Gamage, Kanchana K and Rory Kirchner and Moccia, Rob and Cassel, Seth H and Chen, Kuchuan and Wainger, Brian J and Woolf, Clifford J and Eggan, Kevin}
}
