The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity

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The recognition of ubiquitinated proteins by the proteasome

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Coordination of autophagosome–lysosome fusion and transport by a Klp98A–Rab14 complex inDrosophila

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Gene therapy by proteasome activator, PA28γ, improves motor coordination and proteasome function in Huntington’s disease YAC128 mice

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Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease

(2015) Maria Manczak et al.   HUMAN MOLECULAR GENETICS

Detection of ubiquitinated huntingtin species in intracellular aggregates

(2015) Katrin Juenemann et al.   Frontiers in Molecular Neuroscience

The Differential Profiling of Ubiquitin-Proteasome and Autophagy Systems in Different Tissues before the Onset of Huntington's Disease Models

(2014) Lu-Shiun Her et al.   BRAIN PATHOLOGY

Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1 

(2014) A. Hyrskyluoto et al.   HUMAN MOLECULAR GENETICS

Ubiquitin-Activating Enzyme Activity Contributes to Differential Accumulation of Mutant Huntingtin in Brain and Peripheral Tissues

(2014) B. E. Wade et al.   JOURNAL OF NEUROSCIENCE

Proteasome Failure Promotes Positioning of Lysosomes around the Aggresome via Local Block of Microtubule-Dependent Transport

(2014) N. Zaarur et al.   MOLECULAR AND CELLULAR BIOLOGY

Huntington disease: natural history, biomarkers and prospects for therapeutics

(2014) Christopher A. Ross et al.   Nature Reviews Neurology

Ubiquilin-1 Overexpression Increases the Lifespan and Delays Accumulation of Huntingtin Aggregates in the R6/2 Mouse Model of Huntington's Disease

(2014) Nathaniel Safren et al.   PLoS One

Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A

(2014) K. P. Bhat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ubiquitin–proteasome system involvement in Huntington’s disease

(2014) Zaira Ortega et al.   Frontiers in Molecular Neuroscience

The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

(2014) Nico P. Dantuma et al.   Frontiers in Molecular Neuroscience

miR-196a Ameliorates Phenotypes of Huntington Disease in Cell, Transgenic Mouse, and Induced Pluripotent Stem Cell Models

(2013) Pei-Hsun Cheng et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Expanded Polyglutamine-containing N-terminal Huntingtin Fragments Are Entirely Degraded by Mammalian Proteasomes

(2013) Katrin Juenemann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Functions of the 19S complex in proteasomal degradation

(2013) Chang-Wei Liu et al.   TRENDS IN BIOCHEMICAL SCIENCES

Guidelines for the use and interpretation of assays for monitoring autophagy

(2012) Daniel J. Klionsky et al.   Autophagy

Significantly differential diffusion of neuropathological aggregates in the brain of transgenic mice carrying N-terminal mutant huntingtin fused with green fluorescent protein

(2012) Pei-Hsun Cheng et al.   Brain Structure & Function

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington’s disease

(2012) Mark S. Hipp et al.   JOURNAL OF CELL BIOLOGY

Polyglutamine tracts as modulators of transcriptional activation from yeast to mammals

(2011) Lilit Atanesyan et al.   BIOLOGICAL CHEMISTRY

PARP-1 cleavage fragments: signatures of cell-death proteases in neurodegeneration

(2011) Ganta Chaitanya et al.   Cell Communication and Signaling

Protein aggregates in Huntington's disease

(2011) Montserrat Arrasate et al.   EXPERIMENTAL NEUROLOGY

Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis

(2011) Surya A. Reis et al.   HUMAN MOLECULAR GENETICS

The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease

(2011) Ignacio Munoz-Sanjuan et al.   JOURNAL OF CLINICAL INVESTIGATION

Clearance of mutant huntingtin

(2010) Xiao-Jiang Li et al.   Autophagy

Functional differences between two major ubiquitin receptors in the proteasome; S5a and hRpn13

(2010) Muthukumar Elangovan et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Inhibiting the ubiquitin–proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments

(2010) Xiang Li et al.   HUMAN MOLECULAR GENETICS

Modulation of Mutant Huntingtin N-Terminal Cleavage and Its Effect on Aggregation and Cell Death

(2010) Katrin Juenemann et al.   NEUROTOXICITY RESEARCH

Recognition and Processing of Ubiquitin-Protein Conjugates by the Proteasome

(2009) Daniel Finley   Annual Review of Biochemistry

Intranuclear Degradation of Polyglutamine Aggregates by the Ubiquitin-Proteasome System

(2009) Atsushi Iwata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mimicking proteasomal release of polyglutamine peptides initiates aggregation and toxicity

(2009) M. Raspe et al.   JOURNAL OF CELL SCIENCE

Lysosome biogenesis and lysosomal membrane proteins: trafficking meets function

(2009) Paul Saftig et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis†

(2008) Cristina Cheroni et al.   HUMAN MOLECULAR GENETICS

Protein misfolding inside cells: The case of huntingtin and Huntington's disease

(2008) Danny M. Hatters   IUBMB LIFE

Impaired ubiquitin–proteasome system activity in the synapses of Huntington's disease mice

(2008) Jianjun Wang et al.   JOURNAL OF CELL BIOLOGY

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

(2008) Chuan-En Wang et al.   JOURNAL OF CELL BIOLOGY

Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease

(2008) B. Ravikumar et al.   JOURNAL OF CELL SCIENCE

Calcineurin is involved in the early activation of NMDA-mediated cell death in mutant huntingtin knock-in striatal cells

(2008) Xavier Xifró et al.   JOURNAL OF NEUROCHEMISTRY

Enhanced Sensitivity of Striatal Neurons to Axonal Transport Defects Induced by Mutant Huntingtin

(2008) L.-S. Her et al.   JOURNAL OF NEUROSCIENCE

Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice

(2008) M. Gray et al.   JOURNAL OF NEUROSCIENCE

Proteasome subunit Rpn13 is a novel ubiquitin receptor

(2008) Koraljka Husnjak et al.   NATURE