A novel small molecule HSP90 inhibitor, NXD30001, differentially induces heat shock proteins in nervous tissue in culture and in vivo

Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy

(2013) Bilal Malik et al.   BRAIN

Therapeutic Potential of HSP90 Inhibition for Neurofibromatosis Type 2

(2013) K. Tanaka et al.   CLINICAL CANCER RESEARCH

Mutations in the gene encoding p62 in Japanese patients with amyotrophic lateral sclerosis

(2013) M. Hirano et al.   NEUROLOGY

Detection of a novel frameshift mutation and regions with homozygosis within ARHGEF28 gene in familial amyotrophic lateral sclerosis

(2013) Cristian A. Droppelmann et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Impaired proteasome function in sporadic amyotrophic lateral sclerosis

(2012) Edor Kabashi et al.   Amyotrophic Lateral Sclerosis

Mitochondrial Dynamics and Bioenergetic Dysfunction Is Associated with Synaptic Alterations in Mutant SOD1 Motor Neurons

(2012) J. Magrane et al.   JOURNAL OF NEUROSCIENCE

Hsp72 preserves muscle function and slows progression of severe muscular dystrophy

(2012) Stefan M. Gehrig et al.   NATURE

Mutation analysis of PFN1 in familial amyotrophic lateral sclerosis patients

(2012) Hussein Daoud et al.   NEUROBIOLOGY OF AGING

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α

(2012) Wenjun Song et al.   NEUROBIOLOGY OF DISEASE

Normal role of the low-molecular-weight neurofilament protein in mitochondrial dynamics and disruption in Charcot-Marie-Tooth disease

(2011) Benoit J. Gentil et al.   FASEB JOURNAL

Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease

(2011) John Labbadia et al.   JOURNAL OF CLINICAL INVESTIGATION

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

(2011) Miranda L. Tradewell et al.   NEUROBIOLOGY OF DISEASE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

The Novel Hsp90 Inhibitor NXD30001 Induces Tumor Regression in a Genetically Engineered Mouse Model of Glioblastoma Multiforme

(2010) H. Zhu et al.   MOLECULAR CANCER THERAPEUTICS

Synthesis of pochoxime prodrugs as potent HSP90 inhibitors

(2009) Cuihua Wang et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Inhibition of HSP90 with Pochoximes: SAR and Structure-Based Insights

(2009) Sofia Barluenga et al.   CHEMBIOCHEM

Recent advances in the genetics of amyotrophic lateral sclerosis

(2009) Paul N. Valdmanis et al.   Current Neurology and Neuroscience Reports

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Divergent Synthesis of a Pochonin Library Targeting HSP90 and In Vivo Efficacy of an Identified Inhibitor

(2008) Sofia Barluenga et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

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

Proteasomes remain intact, but show early focal alteration in their composition in a mouse model of amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   JOURNAL OF NEUROCHEMISTRY

Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1G93A mouse model of ALS

(2008) Bernadett Kalmar et al.   JOURNAL OF NEUROCHEMISTRY