Systems biology analysis of Drosophila in vivo screen data elucidates core networks for DNA damage repair in SCA1

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

(2013) Kyota Fujita et al.   Nature Communications

Cell biology of spinocerebellar ataxia

(2012) Harry T. Orr   JOURNAL OF CELL BIOLOGY

Proteomic Investigations Reveal a Role for RNA Processing Factor THRAP3 in the DNA Damage Response

(2012) Petra Beli et al.   MOLECULAR CELL

CAG expansion induces nucleolar stress in polyglutamine diseases

(2012) H. Tsoi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PGC-1  Rescues Huntington's Disease Proteotoxicity by Preventing Oxidative Stress and Promoting TFEB Function

(2012) T. Tsunemi et al.   Science Translational Medicine

Xpa deficiency reduces CAG trinucleotide repeat instability in neuronal tissues in a mouse model of SCA1

(2011) Leroy Hubert et al.   HUMAN MOLECULAR GENETICS

14-3-3 Binding to Ataxin-1(ATXN1) Regulates Its Dephosphorylation at Ser-776 and Transport to the Nucleus

(2011) Shaojuan Lai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human Senataxin Resolves RNA/DNA Hybrids Formed at Transcriptional Pause Sites to Promote Xrn2-Dependent Termination

(2011) Konstantina Skourti-Stathaki et al.   MOLECULAR CELL

Induction and repair of DNA double strand breaks: The increasing spectrum of non-homologous end joining pathways

(2011) Emil Mladenov et al.   MUTATION RESEARCH-FUNDAMENTAL AND MOLECULAR MECHANISMS OF MUTAGENESIS

Determinants and dynamics of genome accessibility

(2011) Oliver Bell et al.   NATURE REVIEWS GENETICS

Ku70 Alleviates Neurodegeneration in Drosophila Models of Huntington's Disease

(2011) Takuya Tamura et al.   PLoS One

Autophagy and polyglutamine diseases

(2011) Maria Jimenez-Sanchez et al.   PROGRESS IN NEUROBIOLOGY

ATM-Dependent Chromatin Changes Silence Transcription In cis to DNA Double-Strand Breaks

(2010) Niraj M. Shanbhag et al.   CELL

Neighborly DISCourse: DNA double strand breaks silence transcription

(2010) Niraj M. Shanbhag et al.   CELL CYCLE

RECQL5 helicase: Connections to DNA recombination and RNA polymerase II transcription

(2010) Ozan Aygün et al.   DNA REPAIR

Suppression of the novel ER protein Maxer by mutant ataxin-1 in Bergman glia contributes to non-cell-autonomous toxicity

(2010) Hiroki Shiwaku et al.   EMBO JOURNAL

Holliday junction-binding activity of human SPF45

(2010) Naoki Horikoshi et al.   GENES TO CELLS

Mutant huntingtin impairs Ku70-mediated DNA repair

(2010) Yasushi Enokido et al.   JOURNAL OF CELL BIOLOGY

The DNA Damage Response: Making It Safe to Play with Knives

(2010) Alberto Ciccia et al.   MOLECULAR CELL

Spinocerebellar Ataxia Type 31 Is Associated with “Inserted” Penta-Nucleotide Repeats Containing (TGGAA)n

(2009) Nozomu Sato et al.   AMERICAN JOURNAL OF HUMAN GENETICS

DNA Damage Regulates Alternative Splicing through Inhibition of RNA Polymerase II Elongation

(2009) Manuel J. Muñoz et al.   CELL

Functional role for senataxin, defective in ataxia oculomotor apraxia type 2, in transcriptional regulation

(2009) Amila Suraweera et al.   HUMAN MOLECULAR GENETICS

Disorders of nucleotide excision repair: the genetic and molecular basis of heterogeneity

(2009) James E. Cleaver et al.   NATURE REVIEWS GENETICS

The Involvement of DNA-Damage and -Repair Defects in Neurological Dysfunction

(2008) Avanti Kulkarni et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Chk1 Is a Histone H3 Threonine 11 Kinase that Regulates DNA Damage-Induced Transcriptional Repression

(2008) Midori Shimada et al.   CELL

Effects of overexpression of Huntingtin proteins on mitochondrial integrity

(2008) Hongmin Wang et al.   HUMAN MOLECULAR GENETICS

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

(2008) Janghoo Lim et al.   NATURE

RNA toxicity is a component of ataxin-3 degeneration in Drosophila

(2008) Ling-Bo Li et al.   NATURE

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE

Transcription-coupled DNA repair: two decades of progress and surprises

(2008) Philip C. Hanawalt et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice

(2008) K. Yamanaka et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

DNA mismatch repair-induced double-strand breaks

(2007) Anetta Nowosielska et al.   DNA REPAIR