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TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis
... Three patients in this study, for example, had un- usually long survival ...their pathology was limited to canonical regions (spinal cord, brainstem, agranular frontal ...different patients with ... See full document
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Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes
... and basal ganglia, including the neos- triatum and globus pallidus from three representative cases of Type 2b were examined using rabbit polyclonal anti-pTDP-43 (phospho Ser409/410) (Cosmo Bio ... See full document
157
Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis
... lated TDP-43 may be identified in the skeletal muscle of both sALS and fALS patients, including patients with and without ...pTDP-43 pathology in ALS. A muscle group-specific ... See full document
22
TDP-43 as a potential biomarker for amyotrophic lateral sclerosis: a systematic review and meta-analysis
... in TDP-43 levels in ALS patients compared to controls, implying that CSF TDP-43 levels show promise as a potential biomarker in ...ALS patients included therein as FTD ... See full document
7
Systemic dysregulation of TDP-43 binding microRNAs in amyotrophic lateral sclerosis
... of TDP-43 binding miRNAs in LCLs of healthy controls and ALS ...ALS patients with no mutation in known ALS ...ALS patients tested irrespective of the genetic ...paralleling ... See full document
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... Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are neurodegenerative disorders, related by signs of deteriorating motor and cognitive functions, and short ...of patients ... See full document
106
Spread of pathology in amyotrophic lateral sclerosis: assessment of phosphorylated TDP-43 along axonal pathways
... ALS patients into four similarly staged groups [8], with corticospinal tract involvement in Stage 1, corticorubral and corticopontine involvement in Stage 2, corticostriatal in Stage 3 and the perforant pathway ... See full document
21
Gamma-synuclein pathology in amyotrophic lateral sclerosis
... four patients, p62- and TDP-43-positive inclusions were more abundant than rarely observed c-synuclein-positive inclusions, suggesting that these are different ... See full document
171
Modeling hallmark pathology using motor neurons derived from the family and sporadic amyotrophic lateral sclerosis patient-specific iPS cells
... Normal TDP-43 is a nuclear protein. Pathological TDP-43, however, is redistributed and sequestered as protein aggre- gates in neuronal nuclei, perikarya, and neurites in the spinal cord and ... See full document
11
Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis
... including TDP-43 and FUS, are genetically linked to ALS and a related condition, frontotemporal lobar degeneration (FTLD) [22 – ...to TDP-43, is required to build paraspeckles [8, 23]. ... See full document
9
Chitotriosidase - a putative biomarker for sporadic amyotrophic lateral sclerosis
... AD patients, presence of glucosamine polymers in amyloid plaques hints at its role in disease pathogenesis ...AD patients [24] underlines the co- Table 1 List of proteins upregulated in ... See full document
6
Tau imaging detects distinctive distribution of tau pathology in ALS/PDC on the Kii Peninsula
... ALS/PDC patients, although it could also be attributed to the tau pathologies in cortical gray matter around the depth of a sulcus and gray–white boundary in the present image analysis, due to relatively low ... See full document
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A blended psychosocial support program for partners of patients with amyotrophic lateral sclerosis and progressive muscular atrophy: protocol of a randomized controlled trial
... Methods: This study is a randomized controlled trial using a wait-list control design. One hundred and forty caregiver-patient dyads, recruited from a nationwide database and through the website of the Dutch ALS Center, ... See full document
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Phosphorylated TDP-43 aggregates in skeletal and cardiac muscle are a marker of myogenic degeneration in amyotrophic lateral sclerosis and various conditions
... spinal anterior horn cells [14]. Based on the above find- ings, we assumed that the maturation process of dense filamentous inclusions is divided into several stages (Fig. 4d, g, j). At first, short-linear aggregates of ... See full document
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Amyotrophic lateral sclerosis mutant TDP 43 may cause synaptic dysfunction through altered dendritic spine function
... in amyotrophic lateral sclerosis (ALS) patients and animal ...protein 43 (TDP-43), the mislocalization and aggregation of which are key pathological features of ... See full document
162
Cognitive and behavioral features of c9FTD/ALS
... The determination of whether memory is impaired varies on the basis of clinical evaluation, neuropsychological testing, and which tests are used. For example, one can consider the older and more simplistic rubric that ... See full document
5
Mice deficient in the C-terminal domain of TAR DNA-binding protein 43 develop age-dependent motor dysfunction associated with impaired Notch1−Akt signaling pathway
... in TDP-ΔC mice, we performed a rotarod test monthly from 2 to 20 months of age ...in TDP-ΔC mice was observed both at 19 and 20 months old ...aged TDP-ΔC mice, we examined the C-boutons, large ... See full document
357
ANXA11 mutations prevail in Chinese ALS patients with and without cognitive dementia
... Chinese patients with ...European patients with ...Chinese patients with SALS respectively, whereas the mutation frequency in Chinese FALS was ...Chinese patients with ALS, ANXA11 is the ... See full document
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Neuropsychological study of amyotrophic lateral sclerosis and parkinsonism-dementia complex in Kii peninsula, Japan
... PSP pathology is characterized by the abnor- mal accumulation of tau protein accompanied by neu- ronal loss and gliosis, mainly in subcortical structures ...PSP patients [30,31]. The cognitive impairment of ... See full document
298
Clinical and multimodal biomarker correlates of ADNI neuropathological findings
... hippocampal sclerosis (HS). In addition, 5 cases had coincident SVD-I. The TDP pathology was confined to amygdala, entorhinal cortex and dentate gyrus in all AD+DLB+MTL-TDP ...group TDP ... See full document
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