DNA-PKcs regulates myogenesis in an Akt-dependent manner independent of induced DNA damage.

Skeletal muscle regeneration relies on muscle stem (satellite) cells. We previously demonstrated that satellite cells efficiently and accurately repair radiation-induced DNA double-strand breaks (DSBs) via the DNA-dependent kinase DNA-PKcs. We show here that DNA-PKcs affects myogenesis independently of its role in DSB repair.

Heterogeneous clinical features in Cockayne syndrome patients and siblings carrying the same CSA mutations.

Background: Cockayne syndrome (CS) is a rare autosomal recessive disorder caused by mutations in ERCC6/CSB or ERCC8/CSA that participate in the transcription-coupled nucleotide excision repair (TC-NER) of UV-induced DNA damage. CS patients display a large heterogeneity of clinical symptoms and severities, the reason of which is not fully understood, and that cannot be anticipated in the diagnostic phase. In addition, little data is available for affected siblings, and this disease is largely undiagnosed in North Africa.

Impaired Knowledge of Social Norms in Dementia and Psychiatric Disorders: Validation of the Social Norms Questionnaire-Dutch Version (SNQ-NL).

The Social Norms Questionnaire-Dutch version (SNQ-NL) measures the ability to understand and identify social boundaries. We examined the psychometric characteristics of the SNQ-NL and its ability to differentiate between patients with behavioral variant frontotemporal dementia (bvFTD; = 23), Alzheimer's dementia (AD; = 26), chronic psychiatric disorders ( = 27), and control participants ( = 92). Between-group differences in the Total score, Break errors, and Overadhere errors were examined and associations with demographic variables and other cognitive functions were explored.

Inhibitory control in trauma-exposed youth: A systematic review.

The aim of this systematic review was to provide insight in inhibitory control (prepotent response inhibition and interference control) in trauma-exposed youth from a developmental perspective and exploring the effects of prolonged stress. A systematic search was conducted, resulting in 1722 abstracts. Of those, 33 studies met inclusion criteria.

CSB promoter downregulation via histone H3 hypoacetylation is an early determinant of replicative senescence.

Cellular senescence has causative links with ageing and age-related diseases, however, it remains unclear if progeroid factors cause senescence in normal cells. Here, we show that depletion of CSB, a protein mutated in progeroid Cockayne syndrome (CS), is the earliest known trigger of p21-dependent replicative senescence. CSB depletion promotes overexpression of the HTRA3 protease resulting in mitochondrial impairments, which are causally linked to CS pathological phenotypes.