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1.

001-es BibID:BIBFORM082386
035-os BibID:(WoS)000528101100002 (Scopus)85084051209
Első szerző:Ashton, Nicholas J.
Cím:An update on blood-based biomarkers for non-Alzheimer neurodegenerative disorders / Nicholas J. Ashton, Abdul Hye, Anto P. Rajkumar, Antoine Leuzy, Stuart Snowden, Marc Suárez-Calvet, Thomas K. Karikari, Michael Schöll, Renaud La Joie, Gil D. Rabinovici, Kina Höglund, Clive Ballard1, Tibor Hortobágyi, Per Svenningsson, Kaj Blennow, Henrik Zetterberg, Dag Aarsland
Dátum:2020
ISSN:1471-003X 1471-0048
Megjegyzések:In recent years, there has been an increasing emphasis on the importance of blood-based biomarkers in the first-in-line evaluation of patients with suspected neurodegenerative disorders (NDD). While neuroimaging (structural and molecular) and cerebrospinal fluid (CSF) analyses identify the underlying pathophysiology at the earliest stage, a biologically relevant marker derived from blood would have greater utility in the primary care setting and in the early screening for eligibility for therapeutic trials. The rapid advancement of ultra-sensitive assays has enabled the investigation of pathological proteins to be measured in blood samples, but research has been predominately focused on Alzheimer's disease (AD) cohorts. Nonetheless, proteins that are currently under scrutiny as blood biomarker candidates for AD (amyloid-?, tau and neurofilament light chain) are likely to have importance for Lewy body dementia's (LBD), frontotemporal dementia's (FTD) and other NDDs in terms of shared pathologies, similar degenerative processes or in the differential diagnosis of clinical symptoms. This review gives an overview and update on the current state of blood-based biomarkers for non-AD NDD, focusing on how candidate AD and novel biomarkers perform in these populations. As background information, we also briefly outline the neuropathological, clinical, molecular imaging and CSF features of the most common NDDs outside of the AD continuum.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Nature Reviews Neuroscience. - 16 : 5 (2020), p. 265-284. -
További szerzők:Hye, Abdul Rajkumar, Anto P. Leuzy, Antoine Snowden, Stuart Suárez-Calvet, Marc Karikari, Thomas K. Schöll, Michael La Joie, Renaud Rabinovici, Gil D. Höglund, Kina Ballard, Clive G. Hortobágyi Tibor (1965-) (patológus) Svenningsson, Per Blennow, Kaj Zetterberg, Henrik Aarsland, Dag
Pályázati támogatás:(2017-1.2.1-NKP-2017-00002)
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2.

001-es BibID:BIBFORM128580
035-os BibID:(Scopus)86000334207 (WoS)001438314000001
Első szerző:Fekete Rebeka
Cím:Microglia dysfunction, neurovascular inflammation and focal neuropathologies are linked to IL-1- and IL-6-related systemic inflammation in COVID-19 / Fekete Rebeka, Simats Alba, Bíró Eduárd, Pósfai Balázs, Cserép Csaba, Schwarcz Anett D., Szabadits Eszter, Környei Zsuzsanna, Tóth Krisztina, Fichó Erzsébet, Szalma János, Vida Sára, Kellermayer Anna, Dávid Csaba, Acsády László, Kontra Levente, Silvestre-Roig Carlos, Moldvay Judit, Fillinger János, Csikász-Nagy Attila, Hortobágyi Tibor, Liesz Arthur, Benkő Szilvia, Dénes Ádám
Dátum:2025
ISSN:1097-6256 1546-1726
Megjegyzések:COVID-19 is associated with diverse neurological abnormalities, but the underlying mechanisms are unclear. We hypothesized that microglia, the resident immune cells of the brain, are centrally involved in this process. To study this, we developed an autopsy platform allowing the integration of molecular anatomy, protein and mRNA datasets in postmortem mirror blocks of brain and peripheral organ samples from cases of COVID-19. We observed focal loss of microglial P2Y12R, CX3CR1?CX3CL1 axis deficits and metabolic failure at sites of virus-associated vascular inflammation in severely affected medullary autonomic nuclei and other brain areas. Microglial dysfunction is linked to mitochondrial injury at sites of excessive synapse and myelin phagocytosis and loss of glutamatergic terminals, in line with proteomic changes of synapse assembly, metabolism and neuronal injury. Furthermore, regionally heterogeneous microglial changes are associated with viral load and central and systemic inflammation related to interleukin (IL)-1 or IL-6 via virus-sensing pattern recognition receptors and inflammasomes. Thus, SARS-CoV-2-induced inflammation might lead to a primarily gliovascular failure in the brain, which could be a common contributor to diverse COVID-19-related neuropathologies.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Diseases of the nervous system
Neuroimmunology
Megjelenés:Nature Neuroscience. - 28 : 3 (2025), p. 558-576. -
További szerzők:Simats, Alba Bíró Eduárd (1998-) (molekuláris biológus) Pósfai Balázs Cserép Csaba Schwarcz, Anett D. Szabadits Eszter Környei Zsuzsanna Tóth Krisztina Fichó Erzsébet Szalma János Vida Sára Kellermayer Anna Dávid Csaba Acsády László Kontra Levente Silvestre-Roig, Carlos Moldvay Judit Fillinger János Csikász-Nagy Attila Hortobágyi Tibor (1965-) (patológus) Liesz, Arthur Benkő Szilvia (1973-) (molekuláris biológus) Dénes Ádám
Pályázati támogatás:K 131844
OTKA
ÚNKP-23-5
Egyéb
ÚNKP-23-4-I
Egyéb
NKFIH SNN132999
Egyéb
ÚNKP-22-3
Egyéb
K 147109
OTKA
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3.

001-es BibID:BIBFORM020101
Első szerző:Görlach, Christoph
Cím:Neuronal nitric oxide synthase inhibitor has a neuroprotective effect in a rat model of brain injury / Christoph Görlach, Tibor Hortobágyi, Szabolcs Hortobágyi, Zoltán Benyó
Dátum:2000
Megjegyzések:Purpose: The aim of the present study was to assess the effects of neuronal nitric oxide synthase (NOS I) inhibitors and a combination of NOS I and NOS II inhibitors on lesion volume after experimental brain injury. Methods: Cold lesion of the brain was induced by application of a precooled (-78 degrees C) copper cylinder to the intact dura of the rat for 6 s. Brains were removed 24 h after the injury and lesion volume determined using the triphenyltetrazolium-chloride method. Results: The specific NOS I inhibitor 3-bromo-7-nitroindazole (Br-7-NI) reduced lesion volume significantly by 21 % compared with the vehicle control. In contrast, 7-nitroindazole had no effect on lesion volume. When aminoguanidine, a specific NOS II inhibitor, was adminis-tered after Br-7-NI, lesion volume was significantly reduced but not significantly more than with either compound alone. Conclusion: Brain injury after cold lesion is partly mediated by NOS I activity and can be attenuated successfully with Br-7-NI, while coin-hibition of NOS II does not improve the outcome significantly.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Restorative Neurology and Neuroscience. - 17 : 2-3 (2000), p. 71-76. -
További szerzők:Hortobágyi Tibor (1965-) (patológus) Hortobágyi Szabolcs Benyó Zoltán
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4.

001-es BibID:BIBFORM020087
Első szerző:Harkány Tibor
Cím:Short-term consequences of N-methyl-D-aspartate excitotoxicity in rat magnocellular nucleus basalis : effects on in vivo labelling of cholinergic neurons / T. Harkany, J. Grosche, J. Mulder, K. M. Horvath, J. Keijser, T. Hortobágyi, P. G. M. Luiten, W. Härtig
Dátum:2001
ISSN:0306-4522
Megjegyzések:Cholinergic neurons of the basal forebrain form one of the neuron populations that are susceptible to excitotoxic injury. Whereas neuropharmacological studies have aimed at rescuing cholinergic neurons from acute excitotoxic attacks, the short-term temporal profile of excitotoxic damage to cholinergic nerve cells remains largely elusive. The effects of N-methyl-D-aspartate (NMDA) infusion on cytochemical markers of cholinergic neurons in rat magnocellular nucleus basalis were therefore determined 4, 24 and 48 h post-lesion. Additionally, the influence of excitotoxic damage on the efficacy of in vivo labelling of cholinergic neurons with carbocyanine 3-192IgG was investigated. Carbocyanine 3-192IgG was unilaterally injected in the lateral ventricle. Twenty-four hours later, NMDA (60 nM/microl) was infused in the right magnocellular nucleus basalis, while control lesions were performed contralaterally. Triple immunofluorescence labelling for carbocyanine 3-192IgG, NMDA receptor 2A and B subunits and choline-acetyltransferase (ChAT) was employed to determine temporal changes in NMDA receptor immunoreactivity on cholinergic neurons. The extent of neuronal degeneration was studied by staining with Fluoro-Jade. Moreover, changes in the numbers of ChAT or p75 low-affinity neurotrophin receptor immunoreactive neurons, and the degree of their co-labelling with carbocyanine 3-192IgG were determined in basal forebrain nuclei. The effects of NMDA-induced lesions on cortical projections of cholinergic nucleus basalis neurons were studied by acetylcholinesterase (AChE) histochemistry. Characteristic signs of cellular damage, as indicated by decreased immunoreactivity for NMDA receptors, ChAT and p75 low-affinity neurotrophin receptors, were already detected at the shortest post-lesion interval investigated. Fluoro-Jade at 4 h post-lesion only labelled the core of the excitotoxic lesion. Longer survival led to enhanced Fluoro-Jade staining, and to the decline of ChAT immunoreactivity reaching a maximum 24 h post-surgery. Significant loss of p75 low-affinity neurotrophin receptor immunoreactivity and of cortical AChE-positive projections only became apparent 48 h post-lesion. Carbocyanine 3-192IgG labelling in the ipsilateral basal forebrain exceeded that of the contralateral hemisphere at all time points investigated and progressively declined in the damaged magnocellular nucleus basalis up to 48 h after NMDA infusion. The present study indicates that excitotoxic lesion-induced alteration of cholinergic neuronal markers is a rapid and gradual process reaching its maximum 24 h post-surgery. Furthermore, in vivo labelling of cholinergic neurons may be applied to indicate neuronal survival under pathological conditions, and enable to follow their degeneration process under a variety of experimental conditions.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Neuroscience. - 108 : 4 (2001), p. 611-627. -
További szerzők:Grosche, Jens Mulder, Jan Horváth Katalin M. Keijser, J. Hortobágyi Tibor (1965-) (patológus) Luiten, Paul G. M. Härtig, Wolfgang
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5.

001-es BibID:BIBFORM020071
Első szerző:Hortobágyi Tibor (patológus)
Cím:Inhibition of neuronal nitric oxide synthase-mediated activation of poly(ADP-ribose) polymerase in traumatic brain injury : neuroprotection by 3-aminobenzamide / T. Hortobágyi, C. Görlach, Z. Benyó, Z. Lacza, S. Hortobágyi, M. Wahl, T. Harkany
Dátum:2003
ISSN:0306-4522
Megjegyzések:Focal traumatic injury to the cerebral cortex is associated with early activation of the neuronal isoform of nitric oxide synthase (nNOS), where high concentrations of nitric oxide-derived free radicals elicit extensive DNA damage. Subsequent activation of the nuclear repair enzyme poly(ADP-ribose) polymerase (PARP) causes a severe energy deficit leading to the ultimate demise of affected neurons. Little is known about the temporal relationship of nNOS and PARP activation and the neuroprotective efficacy of their selective blockade in traumatic brain injury. To determine the relationship of nNOS and PARP activation, brain injury was induced by cryogenic lesion to the somatosensory cortex applying a pre-cooled cylinder after trephination for 6 s to the intact dura mater. Pre-treatment with 3-bromo-7-nitroindazole (BrNI; 25 mg/kg, i.p.), and pre- or combined pre- and post-treatment with 3-aminobenzamide (AB; 10 mg/kg (i.c.v.) or 10 mg/kg/h (i.p.)) were used to inhibit nNOS and PARP, respectively. Cold lesion-induced changes in the somatosensory cortex and neuroprotection by BrNI and AB were determined using immunocytochemistry and immunodot-blot for detection of poly(ADP-ribose; PAR), the end-product of PARP activation, and the triphenyltetrazolium-chloride assay to assess lesion volume. PAR immunoreactivity reached its peak 30 min post-lesion and was followed by gradual reduction of PAR immunolabeling. BrNI pre-treatment significantly decreased the lesion-induced PAR concentration in damaged cerebral cortex. Pre-treatment by i.c.v. infusion of AB markedly diminished cortical PAR immunoreactivity and significantly reduced the lesion volume 24 h post-injury. In contrast, i.p. AB treatment remained largely ineffective. In conclusion, our data indicate early activation of PARP after cold lesion that is, at least in part, related to nNOS induction and supports the relevance of nNOS and/or PARP inhibition to therapeutic approaches of traumatic brain injury.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Neuroscience. - 121 : 4 (2003), p. 983-990. -
További szerzők:Görlach, Christoph Benyó Zoltán Lacza Zsombor Hortobágyi Szabolcs Wahl, Michael Harkány Tibor
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6.

001-es BibID:BIBFORM020293
Első szerző:Hortobágyi Tibor (patológus)
Cím:The significance of diffuse axonal injury : how to diagnose it and what does it tell us? / Tibor Hortobágyi, Safa Al-Sarraj
Dátum:2008
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Advances in clinical neuroscience & rehabilitation. - 8 : 2 (2008), p. 16-18. -
További szerzők:Al-Sarraj, Safa
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7.

001-es BibID:BIBFORM053546
035-os BibID:PMID: 24747576
Első szerző:Huttner, Hagen B.
Cím:The age and genomic integrity of neurons after cortical stroke in humans / Hagen B. Huttner, Olaf Bergmann, Mehran Salehpour, Attila Rácz, Jemal Tatarishvili, Emma Lindgren, Tamás Csonka, László Csiba, Tibor Hortobágyi, Gábor Méhes, Elisabet Englund, Beata Werne Solnestam, Sofia Zdunek, Christian Scharenberg, Lena Ström, Patrik Ståhl, Benjamin Sigurgeirsson, Andreas Dahl, Stefan Schwab, Göran Possnert, Samuel Bernard, Zaal Kokaia, Olle Lindvall, Joakim Lundeberg, Jonas Frisén
Dátum:2014
ISSN:1097-6256
Megjegyzések:It has been unclear whether ischemic stroke induces neurogenesis or neuronal DNA rearrangements in the human neocortex. Using immunohistochemistry; transcriptome, genome and ploidy analyses; and determination of nuclear bomb test-derived (14)C concentration in neuronal DNA, we found neither to be the case. A large proportion of cortical neurons displayed DNA fragmentation and DNA repair a short time after stroke, whereas neurons at chronic stages after stroke showed DNA integrity, demonstrating the relevance of an intact genome for survival.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Nature Neuroscience. - 17 : 6 (2014), p. 801-803. -
További szerzők:Bergmann, Olaf Salehpour, Mehran Rácz Attila (neurológus) Tatarishvili, Jemal Lindgren, Emma Csonka Tamás (1984-) (pathológus) Csiba László (1952-) (neurológus, pszichiáter) Hortobágyi Tibor (1965-) (patológus) Méhes Gábor (1966-) (patológus) Englund, Elisabet Solnestam, Beata Werne Zdunek, Sofia Scharenberg, Christian Ström, Lena Ståhl, Patrik Sigurgeirsson, Benjamin Dahl, Andreas Schwab, Stefan Possnert, Göran Bernard, Samuel Kokaia, Zaal Lindvall, Olle Lundeberg, Joakim Frisén, Jonas
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8.

001-es BibID:BIBFORM082383
Első szerző:Kattuah, Wejdan
Cím:Heterogeneous Nuclear Ribonucleoprotein E2 (hnRNP E2) Is a Component of TDP-43 Aggregates Specifically in the A and C Pathological Subtypes of Frontotemporal Lobar Degeneration / Wejdan Kattuah, Boris Rogelj, Andrew King, Christopher E. Shaw, Tibor Hortobágyi, Claire Troakes
Dátum:2019
ISSN:1662-453X
Megjegyzések:TAR DNA-binding protein 43 (TDP-43) is the major component of the ubiquitin-positive protein aggregates seen in the majority of frontotemporal lobar degeneration and amyotrophic lateral sclerosis cases. TDP-43 belongs to the heterogeneous nuclear ribonucleoprotein (hnRNP) family that is involved in the regulation of RNA transcription, splicing, transport and translation. There are a great many hnRNPs, which often have overlapping functions and act cooperatively in RNA processing. Here we demonstrate that another hnRNP family member, hnRNP E2, shows a striking accumulation within dystrophic neurites and cytoplasmic inclusions in the frontal cortex and hippocampus of a subset of FTLD-TDP cases belonging to pathological subtypes A and C, where hnRNP E2 was found to co-localize with 87% of TDP-43 immunopositive inclusions. hnRNP E2-positive inclusions were not seen in FTLD-TDP cases with the C9orf72 expansion or in any other neurodegenerative disorders examined. This interaction with TDP-43 in specific FTLD subtypes suggests different underlying neurodegenerative pathways.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Frontiers in Neuroscience. - 13 (2019), p. 1-11. -
További szerzők:Rogelj, Boris King, Andrew Shaw, Christopher E. Hortobágyi Tibor (1965-) (patológus) Troakes, Claire
Pályázati támogatás:2017-1.2.1-NKP-2017-00002
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9.

001-es BibID:BIBFORM075780
035-os BibID:(WoS)000442995700011 (Scopus)85052649921
Első szerző:Simándi Zoltán (Ph.D. hallgató, molekuláris biológus)
Cím:Arginine Methyltransferase PRMT8 Provides Cellular Stress Tolerance in Aging Motoneurons / Zoltan Simandi, Krisztian Pajer, Katalin Karolyi, Tatiana Sieler, Lu-Lin Jiang, Zsuzsanna Kolostyak, Zsanett Sari, Zoltan Fekecs, Attila Pap, Andreas Patsalos, Gerardo Alvarado Contreras, Balint Reho, Zoltan Papp, Xiufang Guo, Attila Horvath, Greta Kiss, Zsolt Keresztessy, György Vámosi, James Hickman, Huaxi Xu, Dorothee Dormann, Tibor Hortobagyi, Miklos Antal, Antal Nógrádi, Laszlo Nagy
Dátum:2018
Tárgyszavak:idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:The Journal of neuroscience. - 38 : 35 (2018), p. 7683-7700. -
További szerzők:Pájer Krisztián Károlyi Katalin (pathologia) Sieler, Tatiana Jiang, Lu-Lin Kolostyák Zsuzsanna Sári Zsanett (1991-) (klinikai laboratóriumi kutató) Fekecs Zoltán Pap Attila (1980-) (biológus) Patsalos, Andreas Contreras, Gerardo Alvarado (1978-) (orvos) Rehó Bálint (1992-) Papp Zoltán (1965-) (kardiológus, élettanász) Guo, Xiufang Horváth Attila (1988-) (programtervező informatikus) Kiss Gréta Keresztessy Zsolt Vámosi György (1967-) (biofizikus) Hickman, James Xu, Huaxi Dormann, Dorothee Hortobágyi Tibor (1965-) (patológus) Antal Miklós (1951-) (orvos, anatómus) Nógrádi Antal Nagy László (1966-) (molekuláris sejtbiológus, biokémikus)
Pályázati támogatás:GINOP-2.3.2-15-2016-00043
GINOP
116855
OTKA
124298
OTKA
126885
OTKA
TAMOP 422_2012_0023 VÉD-ELEM
TÁMOP
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10.

001-es BibID:BIBFORM104874
035-os BibID:(scopus)85129606502 (wos)000790633600001
Első szerző:Sonkodi Balázs
Cím:Is the Sex Difference a Clue to the Pathomechanism of Dry Eye Disease? Watch out for the NGF-TrkA-Piezo2 Signaling Axis and the Piezo2 Channelopathy / Sonkodi Balázs, Resch Miklós D., Hortobágyi Tibor
Dátum:2022
ISSN:0895-8696
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Journal Of Molecular Neuroscience. - 72 : 8 (2022), p. 1598-1608. -
További szerzők:Resch Miklós D. Hortobágyi Tibor (1965-) (patológus)
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11.

001-es BibID:BIBFORM037635
Első szerző:Tollervey, James R.
Cím:Characterizing the RNA targets and position-dependent splicing regulation by TDP-43 / Tollervey James R., Curk Tomaz, Rogelj Boris, Briese Michael, Cereda Matteo, Kayikci Melis, König Julian, Hortobágyi Tibor, Nishimura Agnes L., Zupunski Vera, Patani Rickie, Chandran Siddharthan, Rot Gregor, Zupan Blaz, Shaw Christopher E., Ule Jernej
Dátum:2011
ISSN:1097-6256
Megjegyzések:TDP-43 is a predominantly nuclear RNA-binding protein that forms inclusion bodies in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The mRNA targets of TDP-43 in the human brain and its role in RNA processing are largely unknown. Using individual nucleotide-resolution ultraviolet cross-linking and immunoprecipitation (iCLIP), we found that TDP-43 preferentially bound long clusters of UG-rich sequences in vivo. Analysis of RNA binding by TDP-43 in brains from subjects with FTLD revealed that the greatest increases in binding were to the MALAT1 and NEAT1 noncoding RNAs. We also found that binding of TDP-43 to pre-mRNAs influenced alternative splicing in a similar position-dependent manner to Nova proteins. In addition, we identified unusually long clusters of TDP-43 binding at deep intronic positions downstream of silenced exons. A substantial proportion of alternative mRNA isoforms regulated by TDP-43 encode proteins that regulate neuronal development or have been implicated in neurological diseases, highlighting the importance of TDP-43 for the regulation of splicing in the brain.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Nature Neuroscience. - 14 : 4 (2011), p. 452-458. -
További szerzők:Curk, Tomaž Rogelj, Boris Briese, Michael Cereda, Matteo Kayikci, Melis König, Julian Hortobágyi Tibor (1965-) (patológus) Nishimura, Agnes Lumi Župunski, Vera Patani, Rickie Chandran, Siddharthan Rot, Gregor Zupan, Blaž Shaw, Christopher E. Ule, Jernej
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12.

001-es BibID:BIBFORM016353
Első szerző:Tudor, Elizabeth L.
Cím:Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology / Tudor E. L., Galtrey C. M., Perkinton M. S., Lau K. F., De Vos K. J., Mitchell J. C., Ackerley S., Hortobágyi T., Vámos E., Leigh P. N., Klasen C., McLoughlin D. M., Shaw C. E., Miller C. C.
Dátum:2010
ISSN:0306-4522
Megjegyzések:Cytoplasmic ubiquitin-positive inclusions containing TAR-DNA-binding protein-43 (TDP-43) within motor neurons are the hallmark pathology of sporadic amyotrophic lateral sclerosis (ALS). TDP-43 is a nuclear protein and the mechanisms by which it becomes mislocalized and aggregated in ALS are not properly understood. A mutation in the vesicle-associated membrane protein-associated protein-B (VAPB) involving a proline to serine substitution at position 56 (VAPBP56S) is the cause of familial ALS type-8. To gain insight into the molecular mechanisms by which VAPBP56S induces disease, we created transgenic mice that express either wild-type VAPB (VAPBwt) or VAPBP56S in the nervous system. Analyses of both sets of mice revealed no overt motor phenotype nor alterations in survival. However, VAPBP56S but not VAPBwt transgenic mice develop cytoplasmic TDP-43 accumulations within spinal cord motor neurons that were first detected at 18 months of age. Our results suggest a link between abnormal VAPBP56S function and TDP-43 mislocalization.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Neuroscience. - 167 : 3 (2010), p. 774-785. -
További szerzők:Galtrey, C. M. Perkinton, M. S. Lau, K-F. De Vos, Kurt J. Mitchell, Jacqueline C. Ackerley, S. Hortobágyi Tibor (1965-) (patológus) Vámos E. Leigh, P. Nigel Klasen, Christian McLoughlin, D. M. Shaw, Christopher E. Miller, Christopher C.
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