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1 inclusions, recently relabelled the 'TDP-43 proteinopathies'.
2 mice carrying the Amelxp.Y64H mutation is a proteinopathy.
3 ysiological marker of this neurodegenerative proteinopathy.
4 derlying TAR DNA-binding protein 43 (TDP-43) proteinopathy.
5 y confirm the clinical diagnosis of presumed proteinopathy.
6 active response DNA binding protein (TDP-43) proteinopathy.
7 quality control is a risk factor for TDP-43 proteinopathy.
8 may contribute to the pathogenesis of TDP-43 proteinopathy.
9 be a Drosophila melanogaster model of TDP-43 proteinopathy.
10 d cells recapitulates key features of TDP-43 proteinopathy.
11 ensity of the protein and causes multisystem proteinopathy.
12 mitigate disease in a mouse model of TDP-43 proteinopathy.
13 haplotype that is protective against TDP-43 proteinopathy.
14 usly identified protective allele for TDP-43 proteinopathy.
15 cating that mislocated FUS is sufficient for proteinopathy.
16 TDP-43 is predicted to aggravate the TDP-43 proteinopathy.
17 ng new insights into the pathogenesis of FUS proteinopathy.
18 chondrial quality control play a role in FUS proteinopathy.
19 and loss of RNA binding are linked to TDP-43 proteinopathy.
20 ing defect could potentially underlie TDP-43 proteinopathy.
21 n of pathogenic mechanisms underlying TDP-43 proteinopathy.
22 ly unidentified mechanism underlying cardiac proteinopathy.
23 geting pathways downstream of the initiating proteinopathies.
24 active response DNA-binding protein (TDP-43) proteinopathies.
25 nic mechanisms of a group of diseases called proteinopathies.
26 itate its phosphorylation, as seen in TDP-43 proteinopathies.
27 echanisms of disease in ALS and other TDP-43 proteinopathies.
28 Incidence of parkinsonism and specific proteinopathies.
29 a new therapeutic strategy to treat cardiac proteinopathies.
30 parkinsonism, 409 (75.5%) were classified as proteinopathies.
31 moxifen) in a FTLD-U mouse model with TDP-43 proteinopathies.
32 use for neurodegeneration in ALS with TDP-43 proteinopathies.
33 tion of autophagy is protective against some proteinopathies.
34 py of neurodegenerative diseases with TDP-43 proteinopathies.
35 e treatment of Alzheimer's disease and other proteinopathies.
36 noncardiac cells and animal models of other proteinopathies.
37 apeutic intervention in ALS and other TDP-43 proteinopathies.
38 rites are collectively referred to as TDP-43 proteinopathies.
39 n developing therapies for neurodegenerative proteinopathies.
40 ion disease mechanisms underlying the TDP-43 proteinopathies.
41 athology observed in IBMPFD and other TDP-43 proteinopathies.
42 may contribute to the progression of TDP-43 proteinopathies.
43 g a key biochemical characteristic of TDP-43 proteinopathies.
44 f alternative therapeutic targets for TDP-43 proteinopathies.
45 may be a valid therapeutic target for TDP-43 proteinopathies.
46 ic for pathologic inclusions in human TDP-43 proteinopathies.
47 of pathological progression in PD and other proteinopathies.
48 re-overload heart disease in the category of proteinopathies.
49 underlie the development of abnormal TDP-43 proteinopathies.
50 of neurodegenerative diseases called TDP-43 proteinopathies.
51 nts can be utilized to counteract chronic ER proteinopathies.
52 uce the burden of AD and other extracellular proteinopathies.
53 seph disease and possibly other neurological proteinopathies.
54 n microsatellite expansion disorders and RBP proteinopathies.
55 a widely observed event in neurodegenerative proteinopathies.
56 s is crucial for proteostasis and to prevent proteinopathies.
57 a large class of heritable neurodegenerative proteinopathies.
58 pathogenesis of FTLD-TDP and related TDP-43 proteinopathies.
59 ranulin, may contribute to disease in TDP-43 proteinopathies.
60 be critical for halting or reversing TDP-43 proteinopathies.
61 gical changes and may be applicable to other proteinopathies.
62 d manage neurodegenerative disease and other proteinopathies.
63 ng questions about the pathogenesis of other proteinopathies.
64 d provide new therapeutic avenues for TDP-43 proteinopathies.
65 ing key neuropathological features of TDP-43 proteinopathies.
66 derlying mechanisms of this and other TDP-43 proteinopathies.
67 c strategy for treating ALS and other TDP-43 proteinopathies.
68 that recapitulate several features of TDP-43 proteinopathies.
69 degenerative disorders known as multisystem proteinopathy 1 (MSP1); we further showed that the stoic
70 230 with synucleinopathies, 157 with TDP-43 proteinopathies, 144 with tauopathies and 59 with normal
71 tive mediator of neurodegeneration in TDP-43 proteinopathies, a class of disorder that includes ALS a
72 , cellular deficiencies, or sequestration in proteinopathies all present abnormalities that can be co
73 for mitochondrial dysfunction and the multi-proteinopathies (alpha-synuclein-, APP- and Abeta-aggreg
74 roteomic analyses found that the multisystem proteinopathy/amyotrophic lateral sclerosis proteins, he
75 a promising platform for understanding prion proteinopathies and advancing anti-prion therapeutics.
76 al function is pathogenic in several cardiac proteinopathies and can eventually lead to heart failure
77 n of neurodegenerative diseases as misfolded proteinopathies and delineate a novel path from the mole
78 This can be harnessed for early diagnosis of proteinopathies and for drug/vaccine design against them
80 partners, in the pathogenesis of multisystem proteinopathy and amyotrophic lateral sclerosis supports
81 rNLS8 transgenic (Tg) mouse model of TDP-43 proteinopathy and found striking differences in MN respo
83 overed a previously unknown link between FUS proteinopathy and PINK1/Parkin genes, providing new insi
84 ether the enhancement can rescue a bona fide proteinopathy and protect against ischemia/reperfusion (
85 esult, metals both accumulate in microscopic proteinopathies, and can be deficient in cells or cellul
86 ndicate that AI in Enamp.S55I mice is also a proteinopathy, and based on comparative phenotypic analy
89 n incompletely understood, as most mammalian proteinopathies are only detected after irreversible pro
91 ons in expansion repeat diseases and various proteinopathies associated with ALS and frontotemporal d
92 s and a series of transgenic mouse models of proteinopathies associated with neurodegenerative diseas
93 st evidence that an entire neurodegenerative proteinopathy associated with a robust, lethal motor phe
94 sword indispensable for both physiology and proteinopathy, but thus far its structure remains unknow
95 mutations, we established a model of TDP-43 proteinopathies by expressing fluorescently tagged wild-
96 several cytopathological features of TDP-43 proteinopathy by increasing the turnover of pathologic p
97 nd Alzheimer disease, are well-characterized proteinopathies, cardiac diseases have recently been ass
100 ovide candidate substrates for the spread of proteinopathies causing neurodegeneration, and emerging
102 he majority of the ALS cases are with TDP-43 proteinopathies characterized with TDP-43-positive, ubiq
103 zfeldt-Jakob disease are transmissible brain proteinopathies, characterized by the accumulation of a
106 renewable structures cause distinct types of proteinopathies despite the identical primary structure
108 is, frontotemporal dementia, and multisystem proteinopathy-diseases that are characterized by fibrill
109 enic mutation in TDP-43 and show that TDP-43 proteinopathies do not display an astrocyte non-cell-aut
110 lin reduction might be the cause of multiple proteinopathies due to the accelerating accumulation of
111 gh for spontaneous pathological effects from proteinopathies even though several rare proteinopathies
112 e most prevalent of a large group of related proteinopathies for which there is currently no cure.
114 ding protein 43 (TDP-43, encoded by TARDBP ) proteinopathy has recently been described in ageing and
116 is deposited in the vast majority of TDP-43 proteinopathies, implicating other unknown factors for i
117 pha-synuclein generates multiple patterns of proteinopathies in a group of diseases, such as Parkinso
118 and provides an opportunity to study TDP-43 proteinopathies in human neurons generated from patient
120 ophagy mitigates the pathological effects of proteinopathies in the liver, heart, and skeletal muscle
121 activation positively correlates with TDP-43 proteinopathy in NEFH-tTA/tetO-hTDP-43DeltaNLS mice, spo
122 atrophy, neuronal loss, and disease-specific proteinopathy in the language-dominant (mostly left) hem
123 DEM function in the ERAD protects against ER proteinopathy in vivo and thus represents a potential th
125 d a growing list of diseases known as TDP-43 proteinopathies, in which this protein becomes mislocali
126 1 (SCA1) is a paradigmatic neurodegenerative proteinopathy, in which a mutant protein (in this case,
127 studies confirm that the spectrum of TDP-43 proteinopathies includes most cases of sporadic and fami
128 43 pathology marks a spectrum of multisystem proteinopathies including amyotrophic lateral sclerosis,
130 ve pathogenesis in several neurodegenerative proteinopathies, including Alzheimer and Parkinson disea
131 tes several salient features of human TDP-43 proteinopathies, including conversion from nuclear local
132 atrophy; and 4) TDP-43 lesions in two TDP-43 proteinopathies, including frontotemporal lobar degenera
133 hogenic mechanism of a broad array of TDP-43 proteinopathies, including frontotemporal lobar degenera
134 DC7 as a novel therapeutic target for TDP-43 proteinopathies, including FTLD-TDP and amyotrophic late
135 events that are hallmarks of age-associated proteinopathies, including neurodegenerative disorders s
136 rative diseases collectively named as TDP-43 proteinopathy, including amyotrophic lateral sclerosis (
137 eristic finding in sporadic fused in sarcoma proteinopathies, indicating a multisystem disorder.
138 eins are cytoprotective in neurodegenerative proteinopathies involving protein aggregation; for examp
139 at the onset of cognitive deficits in TDP-43 proteinopathies is independent of TDP-43 inclusions.
144 nfirmed FTLD with tauopathy (n = 31), TDP-43 proteinopathy (n = 49), or AD (n = 26) with antemortem C
145 as therefore been suggested as a therapy for proteinopathies, neurons appear to be less responsive to
146 dates for initiating and perhaps propagating proteinopathies of muscle, brain, motor neuron and bone.
147 ly influences tau pathology, the other major proteinopathy of Alzheimer disease and other tauopathies
149 cumulation, it has been proposed that TDP-43 proteinopathies originate from either a loss-of-function
151 onse element DNA-binding protein 43 (TDP-43) proteinopathy patients while accounting for Alzheimer's
152 ogical and clinical features of human TDP-43 proteinopathy, providing a powerful animal model for thi
161 has special relevance for neurodegenerative proteinopathies, such as Alzheimer disease, Parkinson di
162 nal insufficiency (PFI) has been observed in proteinopathies, such as desmin-related cardiomyopathy,
164 rom proteinopathies even though several rare proteinopathies, surfactant protein A and C deficiencies
165 lation of abnormal proteins including TDP-43 proteinopathy, tauopathy and alpha-synucleinopathy.
166 sues that are uniquely susceptible to TDP-43 proteinopathies.TDP-43 aggregation is linked to various
168 's disease, and frontotemporal dementia, are proteinopathies that are associated with the aggregation
171 on, inclusion body myopathy, and multisystem proteinopathy, this work suggests that autophagic cleara
173 nitive deficits in FTLD-TDP and other TDP-43 proteinopathies; thus, the TDP-25 transgenic mice repres
174 udies of TAR DNA-binding protein 43 (TDP-43) proteinopathies to investigate TDP-43 as a candidate gen
175 ped a Caenorhabditis elegans model of TDP-43 proteinopathies to study the cellular, molecular, and ge
176 norhabditis elegans model of neuronal TDP-43 proteinopathy to specifically interrogate the contributi
177 omarkers; and recognition that the two major proteinopathies underlying AD biomarker changes, amyloid
179 munity participants, we observed that TDP-43 proteinopathy was very common, frequently mixed with pat
180 nstream degenerative cascade in multiple CNS proteinopathies, which could potentially lead to the dev
182 an excellent model of respiratory epithelial proteinopathy with spontaneous pulmonary fibrosis and th
184 roteins (EDEMs) protected against chronic ER proteinopathy without inducing toxicity in a Drosophila
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