Mutant valosin‐containing protein causes a novel type of frontotemporal dementia

Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

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AAA-ATPase p97/Cdc48p, a Cytosolic Chaperone Required for Endoplasmic Reticulum-Associated Protein Degradation

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Monoclonal antibody PHF‐1 recognizes tau protein phosphorylated at serine residues 396 and 404

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A complex of mammalian Ufd1 and Npl4 links the AAA‐ATPase, p97, to ubiquitin and nuclear transport pathways

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Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin–proteasome degradation

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p47 is a cofactor for p97-mediated membrane fusion

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Involvement of Valosin-containing Protein, an ATPase Co-purified with IκBα and 26 S Proteasome, in Ubiquitin-Proteasome-mediated Degradation of IκBα

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Distinct AAA-ATPase p97 complexes function in discrete steps of nuclear assembly

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A Conformation‐ and Phosphorylation‐Dependent Antibody Recognizing the Paired Helical Filaments of Alzheimer's Disease

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Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

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Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

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Frontotemporal dementia

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Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

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Interactions of pathological proteins in neurodegenerative diseases

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TDP-43 in the Ubiquitin Pathology of Frontotemporal Dementia With VCP Gene Mutations

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Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype

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Pathological Heterogeneity of Frontotemporal Lobar Degeneration with Ubiquitin-Positive Inclusions Delineated by Ubiquitin Immunohistochemistry and Novel Monoclonal Antibodies

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The genetics and neuropathology of frontotemporal lobar degeneration

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FTD and ALS: A Tale of Two Diseases

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Valosin-containing protein disease: Inclusion body myopathy with Paget’s disease of the bone and fronto-temporal dementia

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TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease

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Novel Ubiquitin Neuropathology in Frontotemporal Dementia WithValosin-Containing ProteinGene Mutations

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The mitochondrial pathway in yeast apoptosis

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VCP disease associated with myopathy, Paget disease of bone and frontotemporal dementia: Review of a unique disorder

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Bridging biophysics and neurology: aberrant phase transitions in neurodegenerative disease

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Autophagy and ALS: mechanistic insights and therapeutic implications

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Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

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Clinical studies in familial VCP myopathy associated with Paget disease of bone and frontotemporal dementia

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Pathological consequences of VCP mutations on human striated muscle

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Loss of TMEM106B Ameliorates Lysosomal and Frontotemporal Dementia-Related Phenotypes in Progranulin-Deficient Mice

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An arginine/lysine‐rich motif is crucial for VCP/p97‐mediated modulation of ataxin‐3 fibrillogenesis

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Valosin-containing protein gene mutations

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A novel ATP‐dependent conformation in p97 N–D1 fragment revealed by crystal structures of disease‐related mutants

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Impaired Protein Aggregate Handling and Clearance Underlie the Pathogenesis of p97/VCP-associated Disease

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Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation

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The Multiple Faces of Valosin-Containing Protein-Associated Diseases: Inclusion Body Myopathy with Paget’s Disease of Bone, Frontotemporal Dementia, and Amyotrophic Lateral Sclerosis

Nalbandian, A., Donkervoort, S. C., Dec, E., Badadani, M., Katheria, V., Rana, P., Nguyen, C. C., Mukherjee, J., Caiozzo, V. J., Martin, B., et al. (2011) · Journal of Molecular Neuroscience · doi:10.1007/s12031-011-9627-y · Cited by 140

TDP-43 Proteinopathies: Neurodegenerative Protein Misfolding Diseases without Amyloidosis

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TDP-43: a novel neurodegenerative proteinopathy

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VCP Associated Inclusion Body Myopathy and Paget Disease of Bone Knock-In Mouse Model Exhibits Tissue Pathology Typical of Human Disease

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The ubiquitin-proteasome system in cardiac proteinopathy: a quality control perspective

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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

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Genotype–phenotype studies of VCP ‐associated inclusion body myopathy with Paget disease of bone and/or frontotemporal dementia

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Inclusion body myopathy and Paget disease is linked to a novel mutation in the VCP gene

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Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice

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Gene expression analysis of frontotemporal lobar degeneration of the motor neuron disease type with ubiquitinated inclusions

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Mutations in the Human AAA+ Chaperone p97 and Related Diseases

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Mechanisms of Cdc48/VCP-mediated cell death — from yeast apoptosis to human disease

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The triple power of D³: Protein intrinsic disorder in degenerative diseases

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Enhanced ATPase activities as a primary defect of mutant valosin‐containing proteins that cause inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia

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Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: common pathways in neurodegenerative disease

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The AAA+ chaperone VCP disaggregates Tau fibrils and generates aggregate seeds in a cellular system

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Crucial Mitochondrial Impairment upon CDC48 Mutation in Apoptotic Yeast

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AAA ATPase p97/VCP: cellular functions, disease and therapeutic potential

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VCP and ATL1 regulate endoplasmic reticulum and protein synthesis for dendritic spine formation

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The molecular genetics and neuropathology of frontotemporal lobar degeneration: recent developments

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Valosin-containing protein and neurofibromin interact to regulate dendritic spine density

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TDP-43 pathology: From noxious assembly to therapeutic removal

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Strumpellin is a novel valosin-containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases

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Impaired protein degradation in FTLD and related disorders

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Inclusion body myopathy and frontotemporal dementia caused by a novel VCP mutation

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Frontotemporal Lobar Degeneration: Current Concepts in the Light of Recent Advances

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The Homozygote VCPR155H/R155H Mouse Model Exhibits Accelerated Human VCP-Associated Disease Pathology

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Frontotemporal dementia

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Genetics and aetiology of Pagetic disorders of bone

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Valosin-Containing Protein (VCP/p97) Is Required for Poliovirus Replication and Is Involved in Cellular Protein Secretion Pathway in Poliovirus Infection

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Characterization of the Asian myopathy patients with VCP mutations

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The Slow Wallerian Degeneration Protein, WldS, Binds Directly to VCP/p97 and Partially Redistributes It within the Nucleus

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Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?

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Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy

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Towards Unveiling the Genetics of Neurodegenerative Diseases

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Inclusion Body Myopathy With Paget Disease of Bone and Frontotemporal Dementia Linked to VCP p.Arg155Cys in a Korean Family

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N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation

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Clinicopathologic correlation in PGRN mutations

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The genetics of frontotemporal lobar degeneration

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Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy

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Hereditary inclusion-body myopathies

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Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant

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Characterization of Ubiquitinated Intraneuronal Inclusions in a Novel Belgian Frontotemporal Lobar Degeneration Family

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Regulated cell death and its role in Alzheimer’s disease and amyotrophic lateral sclerosis

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An Italian family with inclusion‐body myopathy and frontotemporal dementia due to mutation in the VCP gene

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Valosin Containing Protein Associated Fronto-Temporal Lobar Degeneration: Clinical Presentation, Pathologic Features and Pathogenesis

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Evolutionary divergence of valosin‐containing protein/cell division cycle protein 48 binding interactions among endoplasmic reticulum‐associated degradation proteins

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Weighted Protein Interaction Network Analysis of Frontotemporal Dementia

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