scholarly article | Q13442814 |
P50 | author | Conrad C Weihl | Q114404424 |
P2093 | author name string | Alan Pestronk | |
Sara E Miller | |||
Phyllis I Hanson | |||
P2860 | cites work | Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin-proteasome degradation | Q24291519 |
Limb-girdle muscular dystrophy type 2H associated with mutation in TRIM32, a putative E3-ubiquitin-ligase gene | Q24563710 | ||
AAA-ATPase p97/Cdc48p, a cytosolic chaperone required for endoplasmic reticulum-associated protein degradation | Q27939982 | ||
Clinical delineation and localization to chromosome 9p13.3-p12 of a unique dominant disorder in four families: hereditary inclusion body myopathy, Paget disease of bone, and frontotemporal dementia | Q28212105 | ||
Caveolinopathies: mutations in caveolin-3 cause four distinct autosomal dominant muscle diseases | Q28246553 | ||
Tubular aggregates are from whole sarcoplasmic reticulum origin: alterations in calcium binding protein expression in mouse skeletal muscle during aging | Q28252007 | ||
Molecular perspectives on p97-VCP: progress in understanding its structure and diverse biological functions | Q28252070 | ||
Protein accumulation and neurodegeneration in the woozy mutant mouse is caused by disruption of SIL1, a cochaperone of BiP | Q28268556 | ||
AAA ATPase p97/valosin-containing protein interacts with gp78, a ubiquitin ligase for endoplasmic reticulum-associated degradation | Q28279098 | ||
Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation | Q28284708 | ||
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein | Q29619232 | ||
Inclusion body myositis-like phenotype induced by transgenic overexpression of beta APP in skeletal muscle | Q34029128 | ||
Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice | Q34415166 | ||
Inclusion-body myositis and myopathies: different etiologies, possibly similar pathogenic mechanisms | Q34915737 | ||
Endoplasmic reticulum stress and unfolded protein response in inclusion body myositis muscle. | Q35083348 | ||
Proteasome inhibition and aggresome formation in sporadic inclusion-body myositis and in amyloid-beta precursor protein-overexpressing cultured human muscle fibers | Q35085041 | ||
Distinct roles for the AAA ATPases NSF and p97 in the secretory pathway | Q35799558 | ||
Molecular pathology and pathogenesis of inclusion-body myositis | Q36231849 | ||
Animal models for muscular dystrophy show different patterns of sarcolemmal disruption. | Q36273863 | ||
E-box sites and a proximal regulatory region of the muscle creatine kinase gene differentially regulate expression in diverse skeletal muscles and cardiac muscle of transgenic mice | Q36562319 | ||
Limb-girdle muscular dystrophy (LGMD-1C) mutants of caveolin-3 undergo ubiquitination and proteasomal degradation. Treatment with proteasomal inhibitors blocks the dominant negative effect of LGMD-1C mutanta and rescues wild-type caveolin-3. | Q42797699 | ||
Autosomal dominant inclusion body myopathy, Paget disease of bone, and frontotemporal dementia | Q44915338 | ||
Interaction of U-box-type ubiquitin-protein ligases (E3s) with molecular chaperones | Q44930825 | ||
Mutant valosin-containing protein causes a novel type of frontotemporal dementia | Q45283163 | ||
Enhanced detection of congo-red-positive amyloid deposits in muscle fibers of inclusion body myositis and brain of Alzheimer's disease using fluorescence technique | Q46088555 | ||
Pathological consequences of VCP mutations on human striated muscle | Q48418017 | ||
Valosin-containing protein gene mutations: clinical and neuropathologic features | Q48491139 | ||
Visualization of dystrophic muscle fibers in mdx mouse by vital staining with Evans blue: evidence of apoptosis in dystrophin-deficient muscle. | Q50756579 | ||
Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin α2-deficient mice | Q57270383 | ||
P433 | issue | 8 | |
P921 | main subject | protein ubiquitination | Q3547638 |
P304 | page(s) | 919-928 | |
P577 | publication date | 2007-02-28 | |
P1433 | published in | Human Molecular Genetics | Q2720965 |
P1476 | title | Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice. | |
P478 | volume | 16 |
Q37610585 | "Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular Disorders |
Q46501146 | 215th ENMC International Workshop VCP-related multi-system proteinopathy (IBMPFD) 13-15 November 2015, Heemskerk, The Netherlands |
Q35976721 | A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases |
Q34699001 | A knock-in mouse model for the R120G mutation of αB-crystallin recapitulates human hereditary myopathy and cataracts. |
Q35194634 | A network of RNA and protein interactions in Fronto Temporal Dementia |
Q36565507 | A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse |
Q38003982 | Aggrephagy: selective disposal of protein aggregates by macroautophagy. |
Q28258936 | Altered cofactor regulation with disease-associated p97/VCP mutations |
Q37547167 | De novo prion aggregates trigger autophagy in skeletal muscle |
Q58623082 | Drosophilaand Mouse Models of Hereditary Myopathy Caused by Mutations in VCP/p97 |
Q34020429 | Effects of nonsteroidal anti-inflammatory drugs on amyloid-beta pathology in mouse skeletal muscle |
Q30179386 | Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations |
Q38457811 | FTD and ALS--translating mouse studies into clinical trials |
Q35085137 | Global gene expression profiling in R155H knock-in murine model of VCP disease |
Q36034366 | Global gene profiling of VCP-associated inclusion body myopathy |
Q28247784 | Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation |
Q24260685 | How citation distortions create unfounded authority: analysis of a citation network |
Q24608911 | Immunization with amyloid-beta attenuates inclusion body myositis-like myopathology and motor impairment in a transgenic mouse model |
Q36949378 | Impaired protein aggregate handling and clearance underlie the pathogenesis of p97/VCP-associated disease |
Q46148248 | In vitro studies in VCP-associated multisystem proteinopathy suggest altered mitochondrial bioenergetics |
Q33871338 | Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy |
Q37261432 | Inclusion body myositis: a view from the Caenorhabditis elegans muscle |
Q43821019 | Increased autophagy accelerates colchicine-induced muscle toxicity |
Q35016908 | MURF2B, a novel LC3-binding protein, participates with MURF2A in the switch between autophagy and ubiquitin proteasome system during differentiation of C2C12 muscle cells |
Q28080241 | Mutations in the Human AAA+ Chaperone p97 and Related Diseases |
Q33737722 | Mutations in valosin-containing protein (VCP) decrease ADP/ATP translocation across the mitochondrial membrane and impair energy metabolism in human neurons |
Q37062875 | 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. |
Q37748586 | New knowledge on critical osteoclast formation and activation pathways from study of rare genetic diseases of osteoclasts: focus on the RANK/RANKL axis. |
Q38006389 | Pathogenesis of Paget disease of bone. |
Q24336201 | Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels |
Q33815368 | Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model |
Q36080410 | Protective role of cell division cycle 48 (CDC48) protein against neurodegeneration via ubiquitin-proteasome system dysfunction during zebrafish development |
Q36034603 | Rodent Models of Amyotrophic Lateral Sclerosis |
Q35758335 | Sarcoglycanopathies: molecular pathogenesis and therapeutic prospects |
Q33916854 | Specific inhibition of p97/VCP ATPase and kinetic analysis demonstrate interaction between D1 and D2 ATPase domains |
Q37776127 | Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers |
Q36677811 | Structural and functional deviations in disease-associated p97 mutants |
Q37089630 | Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease |
Q37228223 | T-Cell-Mediated Inflammatory Myopathies in HIV-Positive Individuals: A Histologic Study of 19 Cases. |
Q34178673 | The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis. |
Q27316347 | The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology |
Q37926529 | The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis. |
Q28308012 | The requirement for Cdc48/p97 in nuclear protein quality control degradation depends on the substrate and correlates with substrate insolubility |
Q61807545 | Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis |
Q48312639 | Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone |
Q64356591 | ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97 |
Q91968846 | Uncoupling of p97 ATPase activity has a dominant negative effect on protein extraction |
Q33721995 | VCP Associated Inclusion Body Myopathy and Paget Disease of Bone Knock-In Mouse Model Exhibits Tissue Pathology Typical of Human Disease |
Q30540665 | VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations |
Q35635102 | Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis |
Q37434920 | Valosin containing protein associated inclusion body myopathy: abnormal vacuolization, autophagy and cell fusion in myoblasts |
Q33589965 | Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease |
Q24306634 | Valosin-containing protein (VCP/p97) is required for poliovirus replication and is involved in cellular protein secretion pathway in poliovirus infection |
Q37452177 | Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia |
Q34890838 | Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration |
Q36853801 | mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy |
Q33556826 | p97/VCP promotes degradation of CRBN substrate glutamine synthetase and neosubstrates |
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