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Article
Open AccessPatient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo
The stereotypical distribution of TAR DNA-binding 43 protein (TDP-43) aggregates in frontotemporal lobar degeneration (FTLD-TDP) suggests that pathological TDP-43 spreads throughout the brain via cell-to-cell ...
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Article
Open AccessNeuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is associated with the accumulation of pathological neuronal and glial intracytoplasmic inclusions as well as accompanying neuron loss. We ex...
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Article
Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to D. The strength of this histo...
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Article
Multimodal evaluation demonstrates in vivo 18F-AV-1451 uptake in autopsy-confirmed corticobasal degeneration
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Article
Open AccessProgression of motor neuron disease is accelerated and the ability to recover is compromised with advanced age in rNLS8 mice
In order to treat progressive paralysis in ALS patients, it is critical to develop a mouse that closely models human ALS in both pathology and also in the timing of these events. We have recently generated new...
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Article
Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43
Accumulation of phosphorylated cytoplasmic TDP-43 inclusions accompanied by loss of normal nuclear TDP-43 in neurons and glia of the brain and spinal cord are the molecular hallmarks of amyotrophic lateral scl...
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Article
Open AccessNovel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins
The RNA/DNA-binding protein, TDP-43, is the key component of ubiquitinated inclusions characteristic of amyotrophic lateral sclerosis (ALS) and the majority of frontotemporal lobar degeneration (FTLD-TDP) refe...
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Article
TDP-43 skeins show properties of amyloid in a subset of ALS cases
Aggregation of TDP-43 proteins to form intracellular inclusions is the primary pathology in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with TDP-43 inclusions (FTLD-TDP). H...
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Article
Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy
Motor neuron disease (MND) may present as an isolated lower motor neuron (LMN) disorder. Although the significance of pathological 43 kDa transactive responsive sequence DNA binding protein (TDP-43) for amyotr...
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Article
The acetylation of tau inhibits its function and promotes pathological tau aggregation
The microtubule associated protein tau promotes neuronal survival through binding and stabilization of MTs. Phosphorylation regulates tau–microtubule interactions and hyperphosphorylation contributes to the ab...
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Article
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases
Ever since the significance of pathological 43-kDa transactivating responsive sequence DNA-binding protein (TDP-43) for human disease has been recognized in amyotrophic lateral sclerosis (ALS) and frontotempor...
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Article
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies
Accumulation of hyperphosphorylated, ubiquitinated and N-terminally truncated TAR DNA-binding protein (TDP-43) is the pathological hallmark lesion in most familial and sporadic forms of FTLD-U and ALS, which c...
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Article
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease
Recently, TDP-43, a 43 kDa nuclear TAR DNA-binding protein, was identified as the major disease protein in frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U), FTLD-U with motor neuron dis...
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Article
Pathological TDP-43 in parkinsonism–dementia complex and amyotrophic lateral sclerosis of Guam
Pathological TDP-43 is the major disease protein in frontotemporal lobar degeneration characterized by ubiquitin inclusions (FTLD-U) with/without motor neuron disease (MND) and in amyotrophic lateral sclerosis...
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Article
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
The rapid confirmation of the initial report by Neumann et al. (Science 314:130–133, 2006) that transactive response (TAR)-DNA-binding protein 43 (TDP-43) is the major disease protein linking frontotemporal lo...
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Article
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration
TDP-43 was recently identified as the major disease protein in neuronal inclusions in frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). TDP-43 becomes redistributed from the nucleu...
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Article
PRECUSOR FORMS OF RAT SMALL INTESTINAL LACTASE
High molecular weight precursor forms of various intestinal brush border enzymes have been demonstrated by pulse-labelling of intestinal explants maintained in culture. In the present study we have examined in vi...
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Article
1254 THE EARLY-ONSET EFFECT OF BILIARY OBSTRUCTION ON CO EXCRETION IN THE RAT
Common bile duct ligation in the adult male Wistar rat increases the rate of total bilirubin formation (TBF) as estimated by the excretion of carbon monoxide (VeCO). This increase is not due to elevation in th...
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Article
665 INTESTINAL REDIFFERENTIATION IN THE INFANT RAT: IN VIVO EVIDENCE OF AN INTRINSIC PROGRAM
The enteric epithelium of the suckling rat undergoes dramatic functional and cytokinetic changes at the time of weaning. Evidence suggesting that this redifferentiation is preprogrammed and intrinsic has been ...
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Article
1667 DETECTION OF GASTRIC CONTENTS IN TRACHEAL FLUID OF PRETERM INFANTS
Aspiration of feedings in critically ill preterm infants requiring mechanical ventilation may prolong their ventilatory course and may contribute to the development of chronic lung disease. While massive aspir...
