Expansions of simple tandem repeats are responsible for almost 50 human diseases, the
majority of which are severe, degenerative, and not currently treatable or preventable. In this …

Epigenetic mechanisms such as DNA methylation and modifications to histone proteins
regulate high-order DNA structure and gene expression. Aberrant epigenetic mechanisms …

The ability of double-stranded DNA to form a triple-helical structure by hydrogen bonding
with a third strand is well established, but the biological functions of these structures remain …

Friedreich's ataxia (FA) is a debilitating, multisystemic disease caused by the depletion of
frataxin (FXN), a mitochondrial iron-sulfur (Fe-S) cluster biogenesis factor. To understand the …

Objective To investigate whether a histone deacetylase inhibitor (HDACi) would be effective
in an in vitro model for the neurodegenerative disease Friedreich ataxia (FRDA) and to …

Transcription stimulates the genetic instability of trinucleotide repeat sequences. However,
the mechanisms leading to transcription-dependent repeat length variation are unclear. We …

Friedreich's ataxia (FRDA), a neurodegenerative disease characterized by ataxia and other
neurological features, affects 1 in 50,000–100,000 individuals in the USA. However, FRDA …

Intensive scientific research devoted in the recent years to understand the molecular
mechanisms or neurodegeneration in spinocerebellar ataxias (SCAs) are identifying new …

Objective: Friedreich ataxia (FA) is the most common ataxia and results from an expanded
GAA repeat in the first intron of FXN. This leads to epigenetic modifications and reduced …

R-loops are atypical, three-stranded nucleic acid structures that contain a stretch of RNA:
DNA hybrids and an unpaired, single stranded DNA loop. R-loops are physiological relevant …