Genomes and Genes
Affiliation: University Hospitals Leuven
- Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) miceSarah Herdewyn
KU Leuven University of Leuven, Department of Neurosciences, Experimental Neurology and Leuven Research Institute for Neuroscience and Disease LIND, Leuven, Belgium
Mol Neurodegener 9:24. 2014..The effect on the enteric and motor phenotype was assessed, both in animals on normal chow or on a jellified fiber deprived diet, aimed at preventing intestinal obstruction...
- Expanded ATXN2 CAG repeat size in ALS identifies genetic overlap between ALS and SCA2P Van Damme
Department of Neurology, University Hospital Leuven, Leuven, Belgium
Neurology 76:2066-72. 2011..Intermediate CAG repeat expansions in ataxin 2 (ATXN2), the causative gene of spinocerebellar ataxia type 2 (SCA2), have been implicated in sporadic ALS. We studied ATXN2 in a large cohort of patients with sporadic and familial ALS...
- Ca(2+)-permeable AMPA receptors and selective vulnerability of motor neuronsL van den Bosch
Laboratory of Neurobiology, Department of Neurology, University of Leuven, Campus Gasthuisberg, Herestraat 49, B 3000 Leuven, Belgium
J Neurol Sci 180:29-34. 2000..This indicates that despite the abundant expression of GluR2, this subunit is excluded from a subset of AMPA receptors and that the activation of these receptors is responsible for the selective motor neuron death...
- Vascular endothelial growth factor counteracts the loss of phospho-Akt preceding motor neurone degeneration in amyotrophic lateral sclerosisM Dewil
Laboratory for Neurobiology, Experimental Neurology, University of Leuven, Campus Gasthuisberg, Leuven, Belgium
Neuropathol Appl Neurobiol 33:499-509. 2007..From these results, we conclude that the loss of phospho-Akt could be involved in motor neurone death in ALS, and that therapies upregulating phospho-Akt thus might be of clinical relevance...
- The occurrence of mutations in FUS in a Belgian cohort of patients with familial ALSP Van Damme
Department of Neurology, Leuven University Hospital, Herestraat 49, 3000 Leuven, Belgium
Eur J Neurol 17:754-6. 2010..Mutations in fused in sarcoma (FUS) were recently identified as a cause of familial amyotrophic lateral sclerosis (ALS). The frequency of occurrence of mutations in FUS in sets of patients with familial ALS remains to be established...
- Tau levels do not influence human ALS or motor neuron degeneration in the SOD1G93A mouseI Taes
Laboratory of Neurobiology and Department of Neurology, University Hospital Gasthuisberg, K U Leuven, Leuven, Belgium
Neurology 74:1687-93. 2010..However, the precise role of tau in motor neuron degeneration remains uncertain...
- Different receptors mediate motor neuron death induced by short and long exposures to excitotoxicityL van den Bosch
Laboratory of Neurobiology, Department of Neurology, University of Leuven, Leuven, Belgium
Brain Res Bull 53:383-8. 2000..dorsal horn neurons was observed after short KA exposures indicating that the selective vulnerability of motor neurons to excitotoxicity is related to the presence of Ca(2+)-permeable AMPA receptors...
- Abnormal intracellular ca(2+)homeostasis and diseaseL Missiaen
Laboratory of Physiology, K U Leuven Campus Gasthuisberg O N, Leuven, Belgium
Cell Calcium 28:1-21. 2000..In some instances, disease-causing mutations or abnormalities provide us with new insights into the cell biology of the various Ca(2+)transporters...
- Excitotoxicity and amyotrophic lateral sclerosisP Van Damme
Neurobiology, Campus Gasthuisberg, Leuven, Belgium
Neurodegener Dis 2:147-59. 2005..All this information could help to develop new and better therapeutic strategies that could protect motor neurons from excitotoxicity...
- Role of heat shock response and Hsp27 in mutant SOD1-dependent cell deathJ Krishnan
Laboratory of Neurobiology, K U Leuven, Campus Gasthuisberg, Leuven, Belgium
Exp Neurol 200:301-10. 2006..These results show that activation of the heat shock response protects cells against mutant SOD1-dependent cell death, but that Hsp27 is not an essential component of the stress response leading to protection...
- Meta-analysis of vascular endothelial growth factor variations in amyotrophic lateral sclerosis: increased susceptibility in male carriers of the -2578AA genotypeD Lambrechts
Center for Transgene Technology and Gene Therapy, KU Leuven, Leuven, Belgium
J Med Genet 46:840-6. 2009..An initial association study reported that VEGF haplotypes conferred increased susceptibility to ALS in humans, but later studies challenged this initial finding...
- Oxidative stress in amyotrophic lateral sclerosisW Robberecht
Department of Neurology, University Hospital Gasthuisberg, University of Leuven, School of Medicine, Belgium
J Neurol 247:I1-6. 2000....
- Genetics of amyotrophic lateral sclerosisW Robberecht
Dept Neurology, University Hospital Gasthuisberg, University of Leuven, Leuven, Belgium
J Neurol 247:VI/2-6. 2000..A genetic component is also thought to at least contribute to the pathogenesis of sporadic ALS. Their identification is now possible thanks to progress in molecular genetics...
- Amyotrophic lateral sclerosis: pathogenesisR H Brown
Department of Neurology, University of Leuven School of Medicine, University Hospital Gasthuisberg, Belgium
Semin Neurol 21:131-9. 2001..Ultimately, it is hoped that insights from these types of studies will improve the prospects for developing meaningful therapies of ALS...
- Evolution of motor and sensory deficits in amyotrophic lateral sclerosis estimated by neurophysiological techniquesP A Theys
Department of Neurology, University Hospital Gasthuisberg, Leuven, Belgium
J Neurol 246:438-42. 1999..Furthermore, the amyotrophic lateral sclerosis disease process seems preferentially to affect MNFCV...
- Crosstalk between astrocytes and motor neurons: what is the message?L van den Bosch
Neurobiology, K U Leuven, Campus Gasthuisberg O and N2, Herestraat 49, Leuven, Belgium
Exp Neurol 211:1-6. 2008..As a consequence, understanding the interactions between motor neurons and astrocytes in health and disease may have important therapeutic implications...
- Differential contribution of the Na(+)-K(+)-2Cl(-) cotransporter NKCC1 to chloride handling in rat embryonic dorsal root ganglion neurons and motor neuronsJ N Chabwine
Department of Molecular and Cell Biology, Katholieke Universiteit Leuven, Leuven, Belgium
FASEB J 23:1168-76. 2009..The high NKCC1 activity in DRGNs is important for maintaining high [Cl(-)](i), whereas lower NKCC1 activity in MNs allows large [Cl(-)](i) variations during neuronal activity...
- D90A heterozygosity in the SOD1 gene is associated with familial and apparently sporadic amyotrophic lateral sclerosisW Robberecht
Department of Neurology and Laboratory for Neurobiology, University Hospital Gasthuisberg, Leuven, Belgium
Neurology 47:1336-9. 1996..We describe two families with ALS and one apparently sporadic ALS patient who are heterozygous for the D90A mutation. One patient had the unusual phenotype of focal nonprogressing motor neuron disease...
- Protective effect of parvalbumin on excitotoxic motor neuron deathL van den Bosch
Neurobiology, Laboratory of Physiology, Campus Gasthuisberg, Herestraat 49, Leuven, Belgium
Exp Neurol 174:150-61. 2002..Overexpression of a high-affinity Ca(2+) buffer such as PV protects the motor neuron from excitotoxicity and this protective effect depends upon the mode of Ca(2+) entry into the cell...
- Genetic markers of ALSW Robberecht
Department of Neurology, University Hospital Leuven, University of Leuven, School of Medicine, Belgium
Amyotroph Lateral Scler Other Motor Neuron Disord 1:S57-9. 2000..Other mutations are under investigation and confirmed results are eagerly awaited...
- Coexistence of CADASIL and Alzheimer's diseaseV Thijs
Department of Neurology, UZ Gasthuisberg, Katholieke Universiteit Leuven, Belgium
J Neurol Neurosurg Psychiatry 74:790-2. 2003..Mutations in the APP, PSEN1, and PSEN2 genes were not identified. Neuropathological findings of Alzheimer's disease may be found in CADASIL patients...
- Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degenerationB Oosthuyse
The Center for Transgene Technology and Gene Therapy, Flanders Interuniversity Institute for Biotechnology, KU Leuven, Leuven, B 3000, Belgium
Nat Genet 28:131-8. 2001..Acute ischemia is known to cause nonselective neuronal death. Our results indicate that chronic vascular insufficiency and, possibly, insufficient Vegf-dependent neuroprotection lead to the select degeneration of motor neurons...
- A case of hemiparesis associated with cranial nerve lesions due to intravascular lymphomatosis(2003:4b)Y De Bruecker
Department of Radiology, University Hospitals, Catholic University of Leuven, Leuven, Belgium
Eur Radiol 13:1753-5. 2003..Intravascular lymphomatosis should be taken into account in the differential diagnosis of repeated cerebral ischemia of unclear aetiology...