Research Topics
| Manuela NeumannSummaryAffiliation: University of Munich Country: Germany Publications
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Publications
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretionSunita S Shankaran
Munich Center for Integrated Protein Science and Adolf Butenandt Institute, Department of Biochemistry, Laboratory for Neurodegenerative Disease Research, Schillerstrasse 44, 80336 Munich, Germany
J Biol Chem 283:1744-53. 2008..Upon reduction of progranulin, neither a major redistribution of TDP-43 nor proteolytic processing to disease-characterizing C-terminal fragments could be observed...- Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degenerationManuela Neumann
Center for Neuropathology and Prion Research, Ludwig Maximilians University, Feodor Lynen Str 23, 81377 Munich, Germany
Acta Neuropathol 113:543-8. 2007..These results argue against a role of these binding partners in the pathogenesis of FTLD-U and emphasize the specificity of TDP-43 as marker for FTLD-U pathology... - TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: protein misfolding diseases without amyloidosisManuela Neumann
Center for Neuropathology and Prion Research, Ludwig Maximilians University of Munich, Feodor Lynen Strasse 23, 81377 Muenchen, Germany
Arch Neurol 64:1388-94. 2007 - TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusionsManuela Neumann
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
J Neuropathol Exp Neurol 66:177-83. 2007..Taken together, these results expand the spectrum of TDP-43 pathology in FTLD-U, suggesting that white matter pathology might contribute to the neurodegenerative process and clinical symptoms in FTLD-U... - Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosisManuela Neumann
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
Science 314:130-3. 2006..TDP-43 represents the common pathologic substrate linking these neurodegenerative disorders... - Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosisLionel M Igaz
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 3600 Spruce St, 3rd Floor, Maloney Bldg, Philadelphia, PA 19104, USA
Am J Pathol 173:182-94. 2008..Therefore, regionally different pathogenic processes may underlie the development of abnormal TDP-43 proteinopathies... - Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathiesKunihiro Uryu
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
J Neuropathol Exp Neurol 67:555-64. 2008..These findings provide further insight into the burden and clinical significance of TDP-43 pathology in disorders other than FTLD-U and amyotrophic lateral sclerosis... - TDP-43 pathologic lesions and clinical phenotype in frontotemporal lobar degeneration with ubiquitin-positive inclusionsMurray Grossman
Department of Neurology, University of Pennsylvania School of Medicine, 2 Gibson, 3400 Spruce St, Philadelphia, PA 19104 4283, USA
Arch Neurol 64:1449-54. 2007..TDP-43 is a major ubiquitinated disease protein in the pathologic condition of frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U)... - Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 ProteinopathiesLionel M Igaz
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, USA
J Biol Chem 284:8516-24. 2009..Thus, our results show that TDP-43 CTF expression recapitulates key biochemical features of pathological TDP-43 and support the hypothesis that the generation of TDP-43 CTFs is an important step in the pathogenesis of FTLD-U and ALS... - Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutationsPeter Kühnlein
Center for Neuropathology and Prion Research, Ludwig Maximilians University, Munich, Feodor Lynen Strasse 23, 81377 Munich, Germany
Arch Neurol 65:1185-9. 2008..This makes TARDBP, the gene encoding for TDP-43, a candidate for genetic screening in ALS... - Clinical and pathological continuum of multisystem TDP-43 proteinopathiesFelix Geser
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
Arch Neurol 66:180-9. 2009.... - Misfolded proteinase K-resistant hyperphosphorylated alpha-synuclein in aged transgenic mice with locomotor deterioration and in human alpha-synucleinopathiesManuela Neumann
Department of Neuropathology, Ludwig Maximilians University, Munich, Germany
J Clin Invest 110:1429-39. 2002..The transgenic mice showed a progressive deterioration of locomotor function. Thus, misfolding and hyperphosphorylation of alphaS may cause dysfunction of affected brain regions... - TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97Gillian P Ritson
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
J Neurosci 30:7729-39. 2010..We suggest that these findings are likely relevant to the pathogenic mechanism of a broad array of TDP-43 proteinopathies, including frontotemporal lobar degeneration and amyotrophic lateral sclerosis... - Pathological heterogeneity of frontotemporal lobar degeneration with ubiquitin-positive inclusions delineated by ubiquitin immunohistochemistry and novel monoclonal antibodiesDeepak M Sampathu
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 3600 Spruce St, 3rd Floor Maloney Bldg, Philadelphia, PA 19104, USA
Am J Pathol 169:1343-52. 2006..Identification of the disease proteins recognized by these mAbs will further advance understanding of molecular substrates of FTLD-U neurodegenerative pathways... - TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron diseaseLinda K Kwong
Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, HUP, Philadelphia, PA, 19104 4283, USA
Acta Neuropathol 114:63-70. 2007.... - Clinical, genetic, and pathologic characteristics of patients with frontotemporal dementia and progranulin mutationsVivianna M Van Deerlin
Department of Pathology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4283, USA
Arch Neurol 64:1148-53. 2007..Patients with frontotemporal dementia due to mutation of progranulin may have a distinct phenotype... - TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysisVivianna M Van Deerlin
Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Lancet Neurol 7:409-16. 2008..Our aim was to investigate whether TARDBP is a candidate disease gene for familial ALS that is not associated with mutations in superoxide dismutase 1 (SOD1)... - Regional distribution of proteinase K-resistant alpha-synuclein correlates with Lewy body disease stageManuela Neumann
Institute of Neuropathology, Laboratory of Alzheimer's and Parkinson's Disease Research, Ludwig Maximilians University, Munich, Germany
J Neuropathol Exp Neurol 63:1225-35. 2004..Variable amounts of neuritic PK-resistant alphaSYN were found in the striatum of all cases. Thus, PK resistance of alphaSYN may be useful for the development of biomarkers of LB diseases... - TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutationsManuela Neumann
Center for Neuropathology and Prion Research, Ludwig Maximilians University, Munich, Germany
J Neuropathol Exp Neurol 66:152-7. 2007..TDP-43 is a common pathologic substrate linking a variety of distinct patterns of FTLD-U pathology caused by different genetic alterations... - Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusionsVivianna M Van Deerlin
1 Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA 2 These authors contributed equally to this work
Nat Genet 42:234-9. 2010..TMEM106B variants also contribute to genetic risk for FTLD-TDP in individuals with mutations in GRN. Our data implicate variants in TMEM106B as a strong risk factor for FTLD-TDP, suggesting an underlying pathogenic mechanism... - Transactive response DNA-binding protein 43 burden in familial Alzheimer disease and Down syndromeCarol F Lippa
Drexel University College of Medicine Philadelphia, Philadelphia, Pennsylvania 19102, USA
Arch Neurol 66:1483-8. 2009..To assess the transactive response DNA-binding protein 43 (TDP-43) burden in familial forms of Alzheimer disease (FAD) and Down syndrome (DS) to determine whether TDP-43 inclusions are also present... - TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLDSigrun Roeber
Center for Neuropathology and Prion Research, Ludwig Maximilians University Munich, Feodor Lynen Str 23, 81377, Munich, Germany
Acta Neuropathol 116:147-57. 2008..The highly consistent clinical and neuropathological phenotype supports the concept that TDP-43-negative FTLD-U should be considered as a new clinicopathological FTLD entity... - TDP-43 immunoreactivity in anoxic, ischemic and neoplastic lesions of the central nervous systemEdward B Lee
Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
Acta Neuropathol 115:305-11. 2008..These findings expand our knowledge of the distribution and localization of TDP-43, and indicate that the TDP-43 inclusions seen in frontotemporal dementias and motor neuron diseases are specific to a neurodegenerative process... - Pathological properties of the Parkinson's disease-associated protein DJ-1 in alpha-synucleinopathies and tauopathies: relevance for multiple system atrophy and Pick's diseaseManuela Neumann
Institute of Neuropathology, Ludwig Maximilians University of Munich, Munich, Germany
Acta Neuropathol (Berl) 107:489-96. 2004..Our results suggest that DJ-1 is up-regulated in reactive astrocytes as well as in neuronal and glial cells with specific alpha-synucleinopathy and tauopathy... - Novel G335V mutation in the tau gene associated with early onset familial frontotemporal dementiaManuela Neumann
Center for Neuropathology and Prion Research, Ludwig Maximilians University, Munich, Germany
Neurogenetics 6:91-5. 2005.... - Cerebral gene expression profiles in sporadic Creutzfeldt-Jakob diseaseWei Xiang
Center for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Munich, Germany
Ann Neurol 58:242-57. 2005..Conspicuously, sCJD brains showed a great similarity with ageing human brains, both in the global expression patterns and in the identified differentially expressed genes... - Dopaminergic midbrain neurons are the prime target for mitochondrial DNA deletionsAndreas Bender
Dept of Neurology, Mitochondrial Neurogenetics, University of Munich, Marchioninistr 15, 81377, Munich, Germany
J Neurol 255:1231-5. 2008..01; ANOVA). This data shows that there is a specific susceptibility of dopaminergic SN neurons to accumulate substantial amounts of mtDNA deletions, regardless of the underlying clinical phenotype... - Structure/function of alpha-synuclein in health and disease: rational development of animal models for Parkinson's and related diseasesPhilipp J Kahle
Department of Biochemistry, Ludwig Maximilians University, Munich, Germany
J Neurochem 82:449-57. 2002 - Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulinDorothee Dormann
Deutsches Zentrum für Neurodegenerative Erkrankungen and Adolf Butenandt Institute, Department of Biochemistry, Ludwig Maximilians University, Munich, Germany
J Neurochem 110:1082-94. 2009..Our findings therefore suggest that caspase-mediated processing generates CTFs of similar biochemical properties as those occurring in nuclear and cytoplasmic deposits of FTLD-U patients independent of PGRN levels... - Creutzfeldt-Jakob disease in a patient with an R208H mutation of the prion protein gene (PRNP) and a 17-kDa prion protein fragmentSigrun Roeber
Center for Neuropathology and Prion Research, , Feodor-Lynen-Srasse 23, 81377, , Germany
Acta Neuropathol (Berl) 109:443-8. 2005..Neuropathological examination of the post mortem brain revealed tau pathology in the hippocampus and entorhinal cortex, as well as ballooned neurons in the cortex, hippocampus and subcortical gray matter... - Hyperphosphorylation and insolubility of alpha-synuclein in transgenic mouse oligodendrocytesPhilipp J Kahle
Laboratory for Alzheimer s and Parkinson s Disease Research, Department of Biochemistry, Ludwig Maximilians University, D 80336 Munich, Germany
EMBO Rep 3:583-8. 2002..Thus, ectopic expression alpha SYN in OLs might initiate salient features of MSA pathology... - TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusionsNigel J Cairns
MRCPath, Department of Pathology and Immunology, Washington University School of Medicine, Campus Box 8118, St Louis, MO 63110, USA
Am J Pathol 171:227-40. 2007.... - Microglial activation mediates neurodegeneration related to oligodendroglial alpha-synucleinopathy: implications for multiple system atrophyNadia Stefanova
Clinical Neurobiology Unit, Neurodegeneration Research Laboratory, Department of Neurology, Innsbruck Medical University, Innsbruck, Austria
Mov Disord 22:2196-203. 2007.... - Oxidative stress in transgenic mice with oligodendroglial alpha-synuclein overexpression replicates the characteristic neuropathology of multiple system atrophyNadia Stefanova
Clinical Department of Neurology, Innsbruck Medical University, Anichstrasse 35 A 6020, Innsbruck, Austria
Am J Pathol 166:869-76. 2005..Our results indicate that combined mitochondrial inhibition and overexpression of oligodendroglial alpha-SYN generates a novel model of MSA that may be useful for evaluating both pathogenesis and treatment strategies... - Neurodegeneration and motor dysfunction in a conditional model of Parkinson's diseaseSilke Nuber
Department of Medical Genetics, University of Tuebingen, D 72076 Tuebingen, Germany
J Neurosci 28:2471-84. 2008..Furthermore, alpha-syn-associated cytotoxicity is independent of filamentous inclusion body formation in our conditional mouse model... - Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar DegenerationNigel J Cairns
Department of Neurology, Washington University School of Medicine, Campus Box 8118, 660 South Euclid Avenue, St Louis, MO, 63110, USA
Acta Neuropathol 114:5-22. 2007..These criteria will be of value to the practicing neuropathologist and provide a foundation for clinical, clinico-pathologic, mechanistic studies and in vivo models of pathogenesis of FTLD... - Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutationsIan R A Mackenzie
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Ann Neurol 61:427-34. 2007..We recently identified TDP-43 as the major pathological protein in sporadic ALS. In this study, we investigated TDP-43 in a larger series of ALS cases (n = 111), including familial cases with and without SOD1 mutations... - Frontotemporal lobar degeneration: demographic characteristics of 353 patientsJulene K Johnson
Department of Neurology, Memory and Aging Center, University of California, San Francisco, 94117, USA
Arch Neurol 62:925-30. 2005..The publication of consensus criteria for FTLD, however, prompted systematic studies. The criteria categorize FTLD into 3 subgroups: frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia... - Tau protein, Abeta42 and S-100B protein in cerebrospinal fluid of patients with dementia with Lewy bodiesBrit Mollenhauer
Department of Neurology, Georg August University Gottingen, Gottingen, Germany
Dement Geriatr Cogn Disord 19:164-70. 2005..We conclude that more specific markers have to be established for the differentiation of these diseases... - An immunohistochemical study of cases of sporadic and inherited frontotemporal lobar degeneration using 3R- and 4R-specific tau monoclonal antibodiesRohan de Silva
Reta Lila Weston Institute of Neurological Studies, University College London, Windeyer Building, 46 Cleveland St, W1T 4JF, London, UK
Acta Neuropathol (Berl) 111:329-40. 2006..The use of such tau isoform specific antibodies may refine pathological criteria underpinning FTLD... - Age-dependent cognitive decline and amygdala pathology in alpha-synuclein transgenic miceChristian Freichel
Pharma Research, F Hoffmann La Roche Ltd, 4070 Basel, Switzerland
Neurobiol Aging 28:1421-35. 2007..Thus, age-dependent fibrillization of alphaSYN in specific cortical regions concomitant with cognitive decline may reflect dementia with Lewy bodies in a transgenic mouse model... - The amyloid-beta (Abeta) peptide pattern in cerebrospinal fluid in Alzheimer's disease: evidence of a novel carboxyterminally elongated Abeta peptidePiotr Lewczuk
Department of Psychiatry and Psychotherapy, University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany
Rapid Commun Mass Spectrom 17:1291-6. 2003..We conclude that SELDI-TOF offers a promising tool for dementia expression pattern profiling using a minute amount of a biological sample...