Research Topics
Species | Marion Maat SchiemanSummaryAffiliation: Leiden University Medical Center Country: The Netherlands Publications
| Collaborators
|
Detail Information
Publications
Glial reactions and the clearance of amyloid beta protein in the brains of patients with hereditary cerebral hemorrhage with amyloidosis-Dutch typeMarion L C Maat-Schieman
Department of Neurology, K5Q 97, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands
Acta Neuropathol 107:389-98. 2004..Abeta sequestration by glial cells may be a factor limiting the levels of neurotoxic soluble Abeta oligomers in HCHWA-D brain...
Neuronal intranuclear and neuropil inclusions for pathological assessment of Huntington's diseaseMarion Maat-Schieman
Department of Neurology, Leiden University Medical Center, The Netherlands
Brain Pathol 17:31-7. 2007..These results indicate that immunohistochemical analysis for HD inclusions facilitates the pathological evaluation of HD and enhances its accuracy...
Hereditary cerebral hemorrhage with amyloidosis-Dutch typeMarion Maat-Schieman
Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands
Neuropathology 25:288-97. 2005..However, the relative importance of cerebral hemorrhages/infarcts, white matter damage and/or other CAA- or Abeta-related factors for cognitive deterioration in HCHWA-D remains to be determined...
Hypocretin and melanin-concentrating hormone in patients with Huntington diseaseAhmad Aziz
Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands
Brain Pathol 18:474-83. 2008..As the number of MCH-expressing neurons was not affected, alterations in MCH signaling are unlikely to have clinical effects in HD patients...
Famous Russian brains: historical attempts to understand intelligenceAlla A Vein
Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2300 RC Leiden, The Netherlands
Brain 131:583-90. 2008..The (re)discovery of the records of the brain autopsies is meaningful, maybe not so much from a neuroanatomical point of view as from a historical perspective...
Small heat shock protein HspB8: its distribution in Alzheimer's disease brains and its inhibition of amyloid-beta protein aggregation and cerebrovascular amyloid-beta toxicityMicha M M Wilhelmus
Department of Neurology and Alzheimer Centre, 830 LKN, Radboud University Nijmegen Medical Centre, P O Box 9101, 6500, Nijmegen, The Netherlands
Acta Neuropathol 111:139-49. 2006..We conclude that HspB8 might play an important role in regulating Abeta aggregation and, therefore, the development of classic SPs in AD and CAA in HCHWA-D...
Inclusion body myositis. Clinical features and clinical course of the disease in 64 patientsUmesh A Badrising
Dept of Neurology, Leiden University Medical Centre, Leiden, The Netherlands
J Neurol 252:1448-54. 2005..IBM has a unique distribution of muscle weakness. Ankylotic contractures are common. We feel that their joint impact on daily functioning is characteristic for the disease...
Abeta is targeted to the vasculature in a mouse model of hereditary cerebral hemorrhage with amyloidosisMartin C Herzig
Department of Cellular Neurology, Hertie-Institute for Clinical Brain Research, , , Germany
Nat Neurosci 7:954-60. 2004....
Evaluation of diagnostic NOTCH3 immunostaining in CADASILSaskia A J Lesnik Oberstein
Department of Clinical Genetics, K5 R, Leiden University Medical Center, P O Box 9600, 2300 RC Leiden, The Netherlands
Acta Neuropathol 106:107-11. 2003..Confirmation by DNA analysis is requisite for positive results, and when there exists high clinical suspicion, also for negative results...
Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placeboUmesh A Badrising
Department of Neurology, Leiden University Medical Center, The Netherlands
Ann Neurol 51:369-72. 2002..Serum creatine kinase activity decreased significantly in the methotrexate group. We conclude that oral methotrexate did not slow down progression of muscle weakness but decreased serum creatine kinase activity...
Orexin loss in Huntington's diseaseAsa Petersen
Department of Physiological Sciences, Section for Neuronal Survival, Lund, Sweden
Hum Mol Genet 14:39-47. 2005..Our results show that the loss of orexin is a novel and potentially very important pathology in HD...
The cerebral beta-amyloid angiopathies: hereditary and sporadicSandy X Zhang-Nunes
Neurology Clinical Trials Unit and MassGeneral Institute for Neurodegenerative Disease, Department of Neurology, Massachusetts General Hospital, Boston 02114, USA
Brain Pathol 16:30-9. 2006....
Cholinergic neuronal defect without cell loss in Huntington's diseaseRuben Smith
Neuronal Survival Unit, Wallenberg Neuroscience Center, Lund University, BMC A10, 221 84 Lund, Sweden
Hum Mol Genet 15:3119-31. 2006..Taken together, our data show that the cholinergic system is dysfunctional in R6/1 and HD patients. Consequently, they provide a rationale for testing of pro-cholinergic drugs in this disease...
Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseasesRemko de Pril
Research Team Molecular Misreading, Netherlands Institute for Brain Research, Amsterdam, The Netherlands
Hum Mol Genet 13:1803-13. 2004..These findings implicate UBB(+1) as an aggravating factor in polyglutamine-induced neurodegeneration, and clearly identify an important role for the ubiquitin-proteasome system in polyglutamine diseases...
Lipoprotein receptor-related protein-1 mediates amyloid-beta-mediated cell death of cerebrovascular cellsMicha M M Wilhelmus
Department of Neurology and Alzheimer Center, Radboud University Nijmegen Medical Centre, P O Box 9101, 6500 HB Nijmegen, The Netherlands
Am J Pathol 171:1989-99. 2007..Although perivascular cells may adapt their A beta internalization capacity to the levels of A beta present, saturated LRP-1/LDLR-mediated uptake of A beta results in degeneration of perivascular cells...
Islet beta-cell area and hormone expression are unaltered in Huntington's diseaseKarl Bacos
Unit of Molecular Metabolism, Department of Experimental Medical Science, Lund University, CRC 91 11, 205 02 Malmo, Sweden
Histochem Cell Biol 129:623-9. 2008..This may lead to a better understanding of HD and provide novel therapeutic targets for symptomatic treatment in HD...
