Increased levels of UCHL1 are a compensatory response to disrupted ubiquitin homeostasis in spinal muscular atrophy and do not represent a viable therapeutic target

Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy

(2014) Thomas M. Wishart et al.   JOURNAL OF CLINICAL INVESTIGATION

How genetic modifiers influence the phenotype of spinal muscular atrophy and suggest future therapeutic approaches

(2013) Brunhilde Wirth et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

The Ubiquitin Proteasome System as a Potential Target for the Treatment of Neurodegenerative Diseases

(2013) Zheng Ying et al.   CURRENT PHARMACEUTICAL DESIGN

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

The E3 ubiquitin ligase mind bomb 1 ubiquitinates and promotes the degradation of survival of motor neuron protein

(2013) Deborah Y. Kwon et al.   MOLECULAR BIOLOGY OF THE CELL

Total Protein Analysis as a Reliable Loading Control for Quantitative Fluorescent Western Blotting

(2013) Samantha L. Eaton 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

Label-free proteomics identifies Calreticulin and GRP75/Mortalin as peripherally accessible protein biomarkers for spinal muscular atrophy

(2013) Chantal A Mutsaers et al.   Genome Medicine

Ubiquitin ligases and beyond

(2012) Ivan Dikic et al.   BMC BIOLOGY

SMN-inducing compounds for the treatment of spinal muscular atrophy

(2012) Monique A Lorson et al.   Future Medicinal Chemistry

Differential Effects of UCHL1 Modulation on Alpha-Synuclein in PD-Like Models of Alpha-Synucleinopathy

(2012) Anna E. Cartier et al.   PLoS One

Spinal muscular atrophy: going beyond the motor neuron

(2012) Gillian Hamilton et al.   TRENDS IN MOLECULAR MEDICINE

Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice

(2011) Deborah Y. Kwon et al.   HUMAN MOLECULAR GENETICS

The Survival of Motor Neuron (SMN) Protein Interacts with the mRNA-Binding Protein HuD and Regulates Localization of Poly(A) mRNA in Primary Motor Neuron Axons

(2011) C. Fallini et al.   JOURNAL OF NEUROSCIENCE

Ubiquitin Homeostasis Is Critical for Synaptic Development and Function

(2011) P.-C. Chen et al.   JOURNAL OF NEUROSCIENCE

The UCHL1 S18Y polymorphism and Parkinson’s disease in a Japanese population

(2011) Katherine W. Snapinn et al.   PARKINSONISM & RELATED DISORDERS

Ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) regulates the level of SMN expression through ubiquitination in primary spinal muscular atrophy fibroblasts

(2010) Shih-Hsien Hsu et al.   CLINICA CHIMICA ACTA

SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophy

(2010) Markus Riessland et al.   HUMAN MOLECULAR GENETICS

Spinal muscular atrophy: mechanisms and therapeutic strategies

(2010) C. L. Lorson et al.   HUMAN MOLECULAR GENETICS

CNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy

(2010) Marco A. Passini et al.   JOURNAL OF CLINICAL INVESTIGATION

Ubiquitination Acutely Regulates Presynaptic Neurotransmitter Release in Mammalian Neurons

(2010) G. V. Rinetti et al.   JOURNAL OF NEUROSCIENCE

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 and Cellular Roles of Ubiquitin-Specific Deubiquitinating Enzymes

(2009) Francisca E. Reyes-Turcu et al.   Annual Review of Biochemistry

Regulation of Synaptic Structure by Ubiquitin C-Terminal Hydrolase L1

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

Inhibition of the Ubiquitin-Proteasome System Prevents Vaccinia Virus DNA Replication and Expression of Intermediate and Late Genes

(2009) P. S. Satheshkumar et al.   JOURNAL OF VIROLOGY

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

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

Spinal muscular atrophy

(2008) Mitchell R Lunn et al.   LANCET

Regulation of SMN Protein Stability

(2008) B. G. Burnett et al.   MOLECULAR AND CELLULAR BIOLOGY