Corticospinal Motor Neurons Are Susceptible to Increased ER Stress and Display Profound Degeneration in the Absence of UCHL1 Function

Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis

(2014) Lijun Wang et al.   NEUROBIOLOGY OF DISEASE

Exome Sequencing Links Corticospinal Motor Neuron Disease to Common Neurodegenerative Disorders

(2014) G. Novarino et al.   SCIENCE

Could Dysregulation of UPS be a Common Underlying Mechanism for Cancer and Neurodegeneration? Lessons from UCHL1

(2013) Javier H. Jara et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

Moving forward in clinical trials for ALS: motor neurons lead the way please

(2013) Barış Genç et al.   DRUG DISCOVERY TODAY

eGFP Expression under UCHL1 Promoter Genetically Labels Corticospinal Motor Neurons and a Subpopulation of Degeneration-Resistant Spinal Motor Neurons in an ALS Mouse Model

(2013) M. V. Yasvoina et al.   JOURNAL OF NEUROSCIENCE

Molecular logic of neocortical projection neuron specification, development and diversity

(2013) Luciano Custo Greig et al.   NATURE REVIEWS NEUROSCIENCE

Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo

(2013) Alexandra Vaccaro et al.   NEUROBIOLOGY OF DISEASE

Wnt Signaling is Altered by Spinal Cord Neuronal Dysfunction in Amyotrophic Lateral Sclerosis Transgenic Mice

(2013) Li Yu et al.   NEUROCHEMICAL RESEARCH

Inhibition of Fast Axonal Transport by Pathogenic SOD1 Involves Activation of p38 MAP Kinase

(2013) Gerardo A. Morfini et al.   PLoS One

Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration

(2013) Kaya Bilguvar et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Circuits for Skilled Reaching and Grasping

(2012) Bror Alstermark et al.   Annual Review of Neuroscience

Ubiquitin C-terminal hydrolase-L1 interacts with adhesion complexes and promotes cell migration, survival, and anchorage independent growth

(2012) Teresa Frisan et al.   FASEB JOURNAL

UCHL1 regulates ubiquitination and recycling of the neural cell adhesion molecule NCAM

(2012) Hilke Wobst et al.   FEBS Journal

AAV2 mediated retrograde transduction of corticospinal motor neurons reveals initial and selective apical dendrite degeneration in ALS

(2012) Javier H. Jara et al.   NEUROBIOLOGY OF DISEASE

SQSTM1 mutations in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2012) E. Rubino et al.   NEUROLOGY

Corticospinal Motor Neurons and Related Subcerebral Projection Neurons Undergo Early and Specific Neurodegeneration in hSOD1G93A Transgenic ALS Mice

(2011) P. H. Ozdinler et al.   JOURNAL OF NEUROSCIENCE

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice

(2011) Rocky G. Gogliotti et al.   NEUROBIOLOGY OF DISEASE

Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis

(2011) P. Tsaytler et al.   SCIENCE

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Rapid, Simple, and Versatile Manufacturing of Recombinant Adeno-Associated Viral Vectors at Scale

(2010) Martin Lock et al.   HUMAN GENE THERAPY

Characterization of Puma-Dependent and Puma-Independent Neuronal Cell Death Pathways following Prolonged Proteasomal Inhibition

(2010) L. P. Tuffy et al.   MOLECULAR AND CELLULAR BIOLOGY

Sublayer-specific microcircuits of corticospinal and corticostriatal neurons in motor cortex

(2010) Charles T Anderson et al.   NATURE NEUROSCIENCE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Ubiquitin carboxyl-terminal hydrolase L1 is required for maintaining the structure and function of the neuromuscular junction

(2010) F. Chen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Regulation of Synaptic Structure by Ubiquitin C-Terminal Hydrolase L1

(2009) A. E. Cartier et al.   JOURNAL OF NEUROSCIENCE

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

(2009) I. Wegorzewska et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

UCHL1 (PGP 9.5): Neuronal biomarker and ubiquitin system protein

(2009) Ian N.M. Day et al.   PROGRESS IN NEUROBIOLOGY

Differential effects of Usp14 and Uch-L1 on the ubiquitin proteasome system and synaptic activity

(2008) B.J. Walters et al.   MOLECULAR AND CELLULAR NEUROSCIENCE