DNA double-strand breaks (DSBs) can be processed by the Mre11-Rad50-Nbs1 (MRN) complex, which is essential to promote ataxia telangiectasia-mutated (ATM) activation. However, the molecular mechanisms linking MRN activity to ATM are not fully understood. Here, using Xenopus laevis egg extract we show that MRN-dependent processing of DSBs leads to the accumulation of short single-stranded DNA oli...

Ataxia is a common neurological syndrome resulting from cerebellar, vestibular or sensory disorders. The recognition and characterisation of sensory ataxia remains a challenge. Cerebellar ataxia is the more common and easier to identify; sensory ataxia is often mistaken for cerebellar ataxia, leading to diagnostic errors and delays. A coherent aetiological work-up is only possible if clinicians...

Episodic ataxia type 1 (EA1) is an autosomal dominant disorder characterized by continuous myokymia and episodic attacks of ataxia. Mutations in the gene KCNA1 that encodes the voltage-gated potassium channel Kv1.1 are responsible for EA1. In several brain areas, Kv1.1 coassembles with Kv1.4, which confers N-type inactivating properties to heteromeric channels. It is therefore likely that the r...

A patient with atypical temporal lobe seizures involving violent behavior was found to exhibit the neuropsychological syndrome described by Luria (1966, 1976) as pathognomonic of lesions in the depths of the left temporal lobe. This syndrome, mainly an audioverbal memory defect with relatively intact phonemic hearing, was evident in spite of an intact intellect.

For the prediction of episodic acidification large uncertainties are connected to climatic variability and its effect on drought conditions and sea-salt episodes. In this study data on 342 hydrological episodes in 25 Swedish streams, sampled over 10 years, have been analyzed using a recently developed episode model. The results demon5 strate that drought is the most important factor modulating ...