Huntingtin protein, mutated in Huntington's disease, is implicated in nucleic acid–mediated
processes, yet the evidence for direct huntingtin–nucleic acid interaction is limited. Here, we …

Expansions of repeat DNA tracts cause> 70 diseases, and ongoing expansions in brains
exacerbate disease. During expansion mutations, single-stranded DNAs (ssDNAs) form …

Cerebellar dysfunction is increasingly recognized as a critical factor in various neurological
diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), and amyotrophic …

The pathological huntingtin (HTT) trinucleotide repeat underlying Huntington disease (HD)
continues to expand throughout life. Repeat length correlates both with earlier age at onset …

A vexing problem in mitochondrial medicine is our limited capacity to evaluate the extent of
brain disease in vivo. This limitation has hindered our understanding of the mechanisms that …

Modifiers of Huntington's disease (HD) include mismatch repair (MMR) genes; however,
their underlying disease-altering mechanisms remain unresolved. Knockout (KO) alleles for …

Cockayne syndrome (CS) is an autosomal recessive disorder of developmental delay,
multiple organ system degeneration and signs of premature ageing. We show here, using …

Traumatic brain injury (TBI) is a risk factor for neurodegeneration, however little is known
about how different neuron types respond to this kind of injury. In this study, we follow …

Huntington's disease (HD) is caused by expansion of the polyglutamine stretch in huntingtin
protein (HTT) resulting in hallmark aggresomes/inclusion bodies (IBs) composed of mutant …

Huntingtin encodes a 3144 amino acid protein, with a polyglutamine repeat tract at the N-
terminus. Expansion of this repeat tract above a pathogenic threshold of 36 repeats is the …