Published in Autophagy on February 22, 2010
Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron (2010) 7.87
Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). Autophagy (2016) 7.05
Selective autophagy mediated by autophagic adapter proteins. Autophagy (2011) 6.88
Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin. J Cell Biol (2010) 5.94
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet (2010) 5.75
The role of autophagy in neurodegenerative disease. Nat Med (2013) 4.46
Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system. Nat Cell Biol (2012) 3.90
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron (2013) 3.63
Reversible inhibitor of p97, DBeQ, impairs both ubiquitin-dependent and autophagic protein clearance pathways. Proc Natl Acad Sci U S A (2011) 3.20
The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks. Nat Struct Mol Biol (2011) 2.23
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97. J Neurosci (2010) 2.05
A comprehensive glossary of autophagy-related molecules and processes (2nd edition). Autophagy (2011) 2.02
The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology. Physiol Rev (2012) 1.95
Hsp90-Cdc37 chaperone complex regulates Ulk1- and Atg13-mediated mitophagy. Mol Cell (2011) 1.91
The N-end rule pathway. Annu Rev Biochem (2012) 1.88
Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy. Hum Mol Genet (2010) 1.75
VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations. Neuron (2013) 1.70
Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations. Nat Cell Biol (2011) 1.69
Covalent and allosteric inhibitors of the ATPase VCP/p97 induce cancer cell death. Nat Chem Biol (2013) 1.65
Imbalances in p97 co-factor interactions in human proteinopathy. EMBO Rep (2010) 1.55
Aggrephagy: selective disposal of protein aggregates by macroautophagy. Int J Cell Biol (2012) 1.52
Cdc48/p97 and Shp1/p47 regulate autophagosome biogenesis in concert with ubiquitin-like Atg8. J Cell Biol (2010) 1.50
Cdc48/p97 promotes degradation of aberrant nascent polypeptides bound to the ribosome. Elife (2013) 1.44
IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation. PLoS One (2016) 1.39
VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease. PLoS One (2010) 1.34
Deficiency of ATP13A2 leads to lysosomal dysfunction, α-synuclein accumulation, and neurotoxicity. J Neurosci (2012) 1.32
The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis. J Cell Sci (2014) 1.32
Proteotoxic crisis, the ubiquitin-proteasome system, and cancer therapy. BMC Biol (2014) 1.29
The ubiquitin-proteasome system and the autophagic-lysosomal system in Alzheimer disease. Cold Spring Harb Perspect Med (2012) 1.27
Structure-activity relationship study reveals ML240 and ML241 as potent and selective inhibitors of p97 ATPase. ChemMedChem (2013) 1.25
AAA ATPase p97/VCP is essential for TRIM21-mediated virus neutralization. Proc Natl Acad Sci U S A (2012) 1.22
Molecules and their functions in autophagy. Exp Mol Med (2012) 1.21
The complexities of p97 function in health and disease. Mol Biosyst (2010) 1.17
Proliferating cell nuclear antigen (PCNA)-binding protein C1orf124 is a regulator of translesion synthesis. J Biol Chem (2012) 1.16
The role of ubiquitin in autophagy-dependent protein aggregate processing. Genes Cancer (2010) 1.15
Neuronal remodeling and apoptosis require VCP-dependent degradation of the apoptosis inhibitor DIAP1. Development (2011) 1.15
The p97/VCP ATPase is critical in muscle atrophy and the accelerated degradation of muscle proteins. EMBO J (2012) 1.15
TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets. Neurotherapeutics (2015) 1.15
The elimination of accumulated and aggregated proteins: a role for aggrephagy in neurodegeneration. Neurobiol Dis (2010) 1.12
The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution. Front Mol Neurosci (2014) 1.10
Inactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in Drosophila. PLoS Genet (2010) 1.08
Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers. Curr Opin Neurol (2010) 1.08
Autophagy and neuronal cell death in neurological disorders. Cold Spring Harb Perspect Biol (2012) 1.08
Mice deficient in Epg5 exhibit selective neuronal vulnerability to degeneration. J Cell Biol (2013) 1.05
Pharmacologic agents targeting autophagy. J Clin Invest (2015) 1.04
Altered intersubunit communication is the molecular basis for functional defects of pathogenic p97 mutants. J Biol Chem (2013) 0.99
Atrogin-1 deficiency promotes cardiomyopathy and premature death via impaired autophagy. J Clin Invest (2014) 0.98
RNA-Seq profiling of spinal cord motor neurons from a presymptomatic SOD1 ALS mouse. PLoS One (2013) 0.98
Altered cofactor regulation with disease-associated p97/VCP mutations. Proc Natl Acad Sci U S A (2015) 0.97
Ubiquitin-specific protease 25 functions in Endoplasmic Reticulum-associated degradation. PLoS One (2012) 0.96
p97-containing complexes in proliferation control and cancer: emerging culprits or guilt by association? Genes Cancer (2010) 0.96
mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy. Hum Mol Genet (2012) 0.96
Histone deacetylase inhibitors increase glucocerebrosidase activity in Gaucher disease by modulation of molecular chaperones. Proc Natl Acad Sci U S A (2012) 0.95
Autophagy in dementias. Brain Pathol (2012) 0.95
Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model. PLoS Genet (2011) 0.94
Spatial expression patterns of autophagy genes in the eye lens and induction of autophagy in lens cells. Mol Vis (2012) 0.94
Role of p97/VCP (Cdc48) in genome stability. Front Genet (2013) 0.94
Autophagosome dynamics in neurodegeneration at a glance. J Cell Sci (2015) 0.94
Cdc48: a swiss army knife of cell biology. J Amino Acids (2013) 0.94
Ubiquitination of the N-terminal region of caveolin-1 regulates endosomal sorting by the VCP/p97 AAA-ATPase. J Biol Chem (2013) 0.93
Valosin-containing protein (VCP/p97) is required for poliovirus replication and is involved in cellular protein secretion pathway in poliovirus infection. J Virol (2012) 0.91
Valosin-containing protein and neurofibromin interact to regulate dendritic spine density. J Clin Invest (2011) 0.91
Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis. Curr Alzheimer Res (2011) 0.91
Structural and functional deviations in disease-associated p97 mutants. J Struct Biol (2012) 0.90
Defective autophagy in spastizin mutated patients with hereditary spastic paraparesis type 15. Brain (2013) 0.90
A Cdc48p-associated factor modulates endoplasmic reticulum-associated degradation, cell stress, and ubiquitinated protein homeostasis. J Biol Chem (2010) 0.90
Culling sick mitochondria from the herd. J Cell Biol (2010) 0.88
SVIP induces localization of p97/VCP to the plasma and lysosomal membranes and regulates autophagy. PLoS One (2011) 0.87
The ubiquitin regulatory X (UBX) domain-containing protein TUG regulates the p97 ATPase and resides at the endoplasmic reticulum-golgi intermediate compartment. J Biol Chem (2011) 0.87
Autophagy protects C. elegans against necrosis during Pseudomonas aeruginosa infection. Proc Natl Acad Sci U S A (2014) 0.87
De novo prion aggregates trigger autophagy in skeletal muscle. J Virol (2013) 0.86
The established and emerging roles of astrocytes and microglia in amyotrophic lateral sclerosis and frontotemporal dementia. Front Cell Neurosci (2015) 0.86
Novel therapeutic approaches for inclusion body myositis. Curr Opin Rheumatol (2010) 0.85
The emerging roles of microRNAs in the pathogenesis of frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum disorders. J Neurogenet (2014) 0.85
Protein interaction profiling of the p97 adaptor UBXD1 points to a role for the complex in modulating ERGIC-53 trafficking. Mol Cell Proteomics (2012) 0.85
Identification of epithelial stromal interaction 1 as a novel effector downstream of Krüppel-like factor 8 in breast cancer invasion and metastasis. Oncogene (2013) 0.85
Endoplasmic reticulum-associated degradation controls cell surface expression of γ-aminobutyric acid, type B receptors. J Biol Chem (2013) 0.84
The type II deiodinase is retrotranslocated to the cytoplasm and proteasomes via p97/Atx3 complex. Mol Endocrinol (2013) 0.84
Mycobacterium tuberculosis induces the miR-33 locus to reprogram autophagy and host lipid metabolism. Nat Immunol (2016) 0.84
VCP/p97 cooperates with YOD1, UBXD1 and PLAA to drive clearance of ruptured lysosomes by autophagy. EMBO J (2016) 0.83
AKT/mTOR and c-Jun N-terminal kinase signaling pathways are required for chrysotile asbestos-induced autophagy. Free Radic Biol Med (2014) 0.83
Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration. J Mol Neurosci (2011) 0.83
Lysosomal two-pore channel subtype 2 (TPC2) regulates skeletal muscle autophagic signaling. J Biol Chem (2014) 0.83
VBP1 facilitates proteasome and autophagy-mediated degradation of MutS homologue hMSH4. FASEB J (2013) 0.83
Role of Epg5 in selective neurodegeneration and Vici syndrome. Autophagy (2013) 0.83
Methylation of the PTPRO gene in human hepatocellular carcinoma and identification of VCP as its substrate. J Cell Biochem (2013) 0.83
Boning up on autophagy: the role of autophagy in skeletal biology. Autophagy (2013) 0.82
VCP-dependent muscle degeneration is linked to defects in a dynamic tubular lysosomal network in vivo. Elife (2015) 0.82
Heteromeric p97/p97R155C complexes induce dominant negative changes in wild-type and autophagy 9-deficient Dictyostelium strains. PLoS One (2012) 0.82
Ring of Change: CDC48/p97 Drives Protein Dynamics at Chromatin. Front Genet (2016) 0.81
Modulating macroautophagy: a neuronal perspective. Future Med Chem (2012) 0.81
A Dynamic molecular basis for malfunction in disease mutants of p97/VCP. Elife (2016) 0.80
From neurodevelopment to neurodegeneration: the interaction of neurofibromin and valosin-containing protein/p97 in regulation of dendritic spine formation. J Biomed Sci (2012) 0.80
Autophagy dysregulation in cell culture and animals models of spinal muscular atrophy. Mol Cell Neurosci (2014) 0.80
RAB24 facilitates clearance of autophagic compartments during basal conditions. Autophagy (2015) 0.79
Atrogin-1 Deficiency Leads to Myopathy and Heart Failure in Zebrafish. Int J Mol Sci (2016) 0.79
Proteasome dysfunction induces muscle growth defects and protein aggregation. J Cell Sci (2014) 0.79
Insights into muscle degeneration from heritable inclusion body myopathies. Front Aging Neurosci (2015) 0.79
Protective role of cell division cycle 48 (CDC48) protein against neurodegeneration via ubiquitin-proteasome system dysfunction during zebrafish development. J Biol Chem (2012) 0.79
Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice. Am J Pathol (2013) 0.79
p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy. J Biol Chem (2007) 21.73
Functions of lysosomes. Annu Rev Physiol (1966) 17.76
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature (2007) 7.74
The ESCRT machinery in endosomal sorting of ubiquitylated membrane proteins. Nature (2009) 6.99
p62 at the crossroads of autophagy, apoptosis, and cancer. Cell (2009) 5.97
HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin. J Biol Chem (2005) 5.89
Role for Rab7 in maturation of late autophagic vacuoles. J Cell Sci (2004) 5.19
Short-lived green fluorescent proteins for quantifying ubiquitin/proteasome-dependent proteolysis in living cells. Nat Biotechnol (2000) 5.14
Autophagy induction and autophagosome clearance in neurons: relationship to autophagic pathology in Alzheimer's disease. J Neurosci (2008) 4.63
Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet (2005) 4.21
Distinct AAA-ATPase p97 complexes function in discrete steps of nuclear assembly. Nat Cell Biol (2001) 3.67
Rab7 is required for the normal progression of the autophagic pathway in mammalian cells. J Cell Sci (2004) 3.66
ESCRT-III dysfunction causes autophagosome accumulation and neurodegeneration. Curr Biol (2007) 3.27
Identification of components of the murine histone deacetylase 6 complex: link between acetylation and ubiquitination signaling pathways. Mol Cell Biol (2001) 3.09
Cdc48 (p97): a "molecular gearbox" in the ubiquitin pathway? Trends Biochem Sci (2006) 2.97
Cdc48/p97 promotes reformation of the nucleus by extracting the kinase Aurora B from chromatin. Nature (2007) 2.84
Conformational changes of the multifunction p97 AAA ATPase during its ATPase cycle. Nat Struct Biol (2002) 2.38
Neurodegenerative lysosomal disorders: a continuum from development to late age. Autophagy (2008) 2.29
Distinct roles for the AAA ATPases NSF and p97 in the secretory pathway. Mol Biol Cell (2003) 2.09
Valosin-containing protein (p97) is a regulator of endoplasmic reticulum stress and of the degradation of N-end rule and ubiquitin-fusion degradation pathway substrates in mammalian cells. Mol Biol Cell (2006) 2.07
VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder. Biochim Biophys Acta (2008) 2.06
VCIP135, a novel essential factor for p97/p47-mediated membrane fusion, is required for Golgi and ER assembly in vivo. J Cell Biol (2002) 2.06
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation. Hum Mol Genet (2005) 2.01
Endoplasmic reticulum stress compromises the ubiquitin-proteasome system. Hum Mol Genet (2005) 1.90
Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease. J Biol Chem (2008) 1.82
Autophagy and the ubiquitin-proteasome system: collaborators in neuroprotection. Biochim Biophys Acta (2008) 1.73
Domain-specific mutations in sequestosome 1 (SQSTM1) cause familial and sporadic Paget's disease. Hum Mol Genet (2002) 1.72
Heat shock and oxygen radicals stimulate ubiquitin-dependent degradation mainly of newly synthesized proteins. J Cell Biol (2008) 1.71
HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration. Autophagy (2007) 1.62
Abeta42-induced neurodegeneration via an age-dependent autophagic-lysosomal injury in Drosophila. PLoS One (2009) 1.61
Clinical studies in familial VCP myopathy associated with Paget disease of bone and frontotemporal dementia. Am J Med Genet A (2008) 1.57
DR5 knockout mice are compromised in radiation-induced apoptosis. Mol Cell Biol (2005) 1.55
Deletion of the SNARE vti1b in mice results in the loss of a single SNARE partner, syntaxin 8. Mol Cell Biol (2003) 1.38
Valosin containing protein associated inclusion body myopathy: abnormal vacuolization, autophagy and cell fusion in myoblasts. Neuromuscul Disord (2009) 1.23
Destabilization of the VCP-Ufd1-Npl4 complex is associated with decreased levels of ERAD substrates. Exp Cell Res (2006) 1.04
ER-associated complexes (ERACs) containing aggregated cystic fibrosis transmembrane conductance regulator (CFTR) are degraded by autophagy. Eur J Cell Biol (2009) 1.01
The essential role of early endosomes in autophagy is revealed by loss of COPI function. Autophagy (2009) 0.92
Regulation of ER-associated degradation via p97/VCP-interacting motif. Biochem Soc Trans (2008) 0.89
Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes. Autophagy (2007) 20.92
Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08
HDAC6 is a microtubule-associated deacetylase. Nature (2002) 12.76
Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron (2010) 7.87
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature (2007) 7.74
HDAC6 regulates Hsp90 acetylation and chaperone-dependent activation of glucocorticoid receptor. Mol Cell (2005) 6.00
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature (2013) 4.03
HDAC6 modulates cell motility by altering the acetylation level of cortactin. Mol Cell (2007) 3.99
HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy. EMBO J (2010) 3.90
Disease-causing mutations in parkin impair mitochondrial ubiquitination, aggregation, and HDAC6-dependent mitophagy. J Cell Biol (2010) 3.37
MDM2-HDAC1-mediated deacetylation of p53 is required for its degradation. EMBO J (2002) 3.29
Polarised asymmetric inheritance of accumulated protein damage in higher eukaryotes. PLoS Biol (2006) 3.06
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol (2007) 2.97
CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome. Neuron (2013) 2.94
HDAC6 controls major cell response pathways to cytotoxic accumulation of protein aggregates. Genes Dev (2007) 2.56
A dynamic ubiquitin equilibrium couples proteasomal activity to chromatin remodeling. J Cell Biol (2006) 2.55
Altered ribostasis: RNA-protein granules in degenerative disorders. Cell (2013) 2.38
Repeat expansion disease: progress and puzzles in disease pathogenesis. Nat Rev Genet (2010) 2.36
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery. J Proteome Res (2010) 2.35
Regulation of MEF2 by histone deacetylase 4- and SIRT1 deacetylase-mediated lysine modifications. Mol Cell Biol (2005) 2.28
Valproic acid increases SMN levels in spinal muscular atrophy patient cells. Ann Neurol (2003) 2.28
Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy. Neuron (2009) 2.26
Heterochromatin protein 1 is recruited to various types of DNA damage. J Cell Biol (2009) 2.26
Histone deacetylase 6 regulates growth factor-induced actin remodeling and endocytosis. Mol Cell Biol (2007) 2.25
The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks. Nat Struct Mol Biol (2011) 2.23
DNA damage triggers nucleotide excision repair-dependent monoubiquitylation of histone H2A. Genes Dev (2006) 2.22
HDAC6 deacetylation of tubulin modulates dynamics of cellular adhesions. J Cell Sci (2007) 2.22
Molecular cloning and characterization of a novel histone deacetylase HDAC10. J Biol Chem (2001) 2.18
The cytoplasmic deacetylase HDAC6 is required for efficient oncogenic tumorigenesis. Cancer Res (2008) 2.17
A genome-wide association study identifies susceptibility loci for nonsyndromic sagittal craniosynostosis near BMP2 and within BBS9. Nat Genet (2012) 2.12
Nutritional phases in Prader-Willi syndrome. Am J Med Genet A (2011) 2.08
VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: review of a unique disorder. Biochim Biophys Acta (2008) 2.06
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97. J Neurosci (2010) 2.05
Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell (2007) 2.01
Cyclin D1 inhibits peroxisome proliferator-activated receptor gamma-mediated adipogenesis through histone deacetylase recruitment. J Biol Chem (2005) 2.01
Intracellular trafficking of histone deacetylase 4 regulates neuronal cell death. J Neurosci (2005) 2.00
A transgenic mouse model of the ubiquitin/proteasome system. Nat Biotechnol (2003) 1.95
Mutant valosin-containing protein causes a novel type of frontotemporal dementia. Ann Neurol (2005) 1.94
Endoplasmic reticulum stress compromises the ubiquitin-proteasome system. Hum Mol Genet (2005) 1.90
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function. Cell (2013) 1.88
Molecular analysis expands the spectrum of phenotypes associated with GLI3 mutations. Hum Mutat (2010) 1.88
Regulation of the dynamics of hsp90 action on the glucocorticoid receptor by acetylation/deacetylation of the chaperone. J Biol Chem (2005) 1.81
Nucleotide excision repair-induced H2A ubiquitination is dependent on MDC1 and RNF8 and reveals a universal DNA damage response. J Cell Biol (2009) 1.77
Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy. Neuron (2010) 1.75
Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation. Hum Mol Genet (2005) 1.73
Autophagy and the ubiquitin-proteasome system: collaborators in neuroprotection. Biochim Biophys Acta (2008) 1.73
Aggregate formation inhibits proteasomal degradation of polyglutamine proteins. Hum Mol Genet (2002) 1.73
The histone deacetylase HDAC4 connects neural activity to muscle transcriptional reprogramming. J Biol Chem (2007) 1.70
VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations. Neuron (2013) 1.70
Pathological consequences of VCP mutations on human striated muscle. Brain (2006) 1.68
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone. Hum Mol Genet (2010) 1.64
HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration. Autophagy (2007) 1.62
Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis. Muscle Nerve (2009) 1.57
A fluorescent broad-spectrum proteasome inhibitor for labeling proteasomes in vitro and in vivo. Chem Biol (2006) 1.54
A conserved unfoldase activity for the p97 AAA-ATPase in proteasomal degradation. J Mol Biol (2009) 1.54
Identification of novel deletions of 15q11q13 in Angelman syndrome by array-CGH: molecular characterization and genotype-phenotype correlations. Eur J Hum Genet (2007) 1.52
Deactylase inhibitors disrupt cellular complexes containing protein phosphatases and deacetylases. J Biol Chem (2003) 1.50
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Hum Mol Genet (2011) 1.47
Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation. J Cell Biol (2002) 1.42
Acetylation goes global: the emergence of acetylation biology. Sci Signal (2009) 1.41
AcK-knowledge reversible acetylation. Sci STKE (2004) 1.39
Polymicrogyria and deletion 22q11.2 syndrome: window to the etiology of a common cortical malformation. Am J Med Genet A (2006) 1.38
The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis. J Mol Neurosci (2011) 1.36
The microtubule-associated histone deacetylase 6 (HDAC6) regulates epidermal growth factor receptor (EGFR) endocytic trafficking and degradation. J Biol Chem (2010) 1.36
DDB2 promotes chromatin decondensation at UV-induced DNA damage. J Cell Biol (2012) 1.34
VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease. PLoS One (2010) 1.34
Deficiency of ATP13A2 leads to lysosomal dysfunction, α-synuclein accumulation, and neurotoxicity. J Neurosci (2012) 1.32
Parathyroid hormone-related peptide represses chondrocyte hypertrophy through a protein phosphatase 2A/histone deacetylase 4/MEF2 pathway. Mol Cell Biol (2009) 1.31
HDAC6 is required for epidermal growth factor-induced beta-catenin nuclear localization. J Biol Chem (2008) 1.31
The UBA2 domain functions as an intrinsic stabilization signal that protects Rad23 from proteasomal degradation. Mol Cell (2005) 1.30
Flightless flies: Drosophila models of neuromuscular disease. Ann N Y Acad Sci (2010) 1.28
The synergistic combination of the farnesyl transferase inhibitor lonafarnib and paclitaxel enhances tubulin acetylation and requires a functional tubulin deacetylase. Cancer Res (2005) 1.28
Disease mutations in Rab7 result in unregulated nucleotide exchange and inappropriate activation. Hum Mol Genet (2009) 1.27
GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Am J Hum Genet (2002) 1.25
Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis. Hum Mol Genet (2008) 1.24
A new non-catalytic role for ubiquitin ligase RNF8 in unfolding higher-order chromatin structure. EMBO J (2012) 1.24
Polyglutamines placed into context. Neuron (2003) 1.21
The role of autophagy in age-related neurodegeneration. Neurosignals (2007) 1.21
Rescue of polyglutamine-mediated cytotoxicity by double-stranded RNA-mediated RNA interference. Hum Mol Genet (2002) 1.21
Removal of Pex3p is an important initial stage in selective peroxisome degradation in Hansenula polymorpha. J Biol Chem (2002) 1.20
Stimulation of GCMa transcriptional activity by cyclic AMP/protein kinase A signaling is attributed to CBP-mediated acetylation of GCMa. Mol Cell Biol (2005) 1.18
Two motifs target Batten disease protein CLN3 to lysosomes in transfected nonneuronal and neuronal cells. Mol Biol Cell (2003) 1.16
Overexpression of the cochaperone CHIP enhances Hsp70-dependent folding activity in mammalian cells. Mol Cell Biol (2003) 1.16
FOXO3a is broadly neuroprotective in vitro and in vivo against insults implicated in motor neuron diseases. J Neurosci (2009) 1.15
Hsp70 dynamics in vivo: effect of heat shock and protein aggregation. J Cell Sci (2004) 1.13
Class IIb HDAC6 regulates endothelial cell migration and angiogenesis by deacetylation of cortactin. EMBO J (2011) 1.12
Dose-dependent inhibition of proteasome activity by a mutant ubiquitin associated with neurodegenerative disease. J Cell Sci (2007) 1.11
Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome. PLoS Genet (2010) 1.11
Proteasomes activate aggresome disassembly and clearance by producing unanchored ubiquitin chains. Mol Cell (2013) 1.10
Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk. J Neurosci (2009) 1.10
Acute polyglutamine expression in inducible mouse model unravels ubiquitin/proteasome system impairment and permanent recovery attributable to aggregate formation. J Neurosci (2010) 1.10