[PDF] Top 20 Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
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Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
... monoclonal TDP-43 anti- body, which was generated using amino acids 1 to 261 of hTDP-43 as the immunogen and was found to recognize amino acids 205-222 of hTDP-43 by epitope mapping [32]; ... See full document
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Dysregulation of the ALS associated gene TDP 43 leads to neuronal death and degeneration in mice
... protein 43 (TDP-43). TDP-43 is normally localized in the nucleus with roles in the regulation of gene expression, and pathological cytoplasmic aggregates are associated with ... See full document
14
CArG elements control smooth muscle subtype–specific expression of smooth muscle myosin in vivo
... genic mice in vivo (12, ...multiple transgenic lines, clearly demonstrating the requirement of the first intronic sequence for expression of the SM-MHC gene in ... See full document
13
Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage
... of TDP-43 in transgenic mice increases expression of inflam- matory markers and TLR2 in glial cells [17, 24]; thus, we hypothesized that increase in TDP-43 levels and the ... See full document
15
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS
... from TDP-43 transgenic mice showed that the exogenous human TDP-43 protein had a slightly higher molecular weight than the en- dogenous mouse TDP-43, due to the ... See full document
16
An investigation into the combined effects of β-amyloid toxicity and cerebral ischemia on the pathological expression of gangliosides.
... APP transgenic mice, as high levels of GM3 in vascular and parenchymal tissues was associated with increased levels of deposited Aβ in vascular tissues (Oikawa et ...GM3 expression is elevated in ... See full document
153
Mitochondrial dynamic abnormalities in amyotrophic lateral sclerosis
... the expression of Miro 1, the only known mitochon- drial outer membrane protein directly coupling mitochon- dria and motor-adaptor complexes, was significantly reduced in spinal cords of ALS patients, strongly ... See full document
6
Znf179 E3 ligase-mediated TDP-43 polyubiquitination is involved in TDP-43- ubiquitinated inclusions (UBI) (+)-related neurodegenerative pathology
... regulating neuronal differentiation ...for neuronal differentiation in cultured cells [25], suggesting its important function during neurogen- ...Znf179 transgenic mice have improved ... See full document
17
Cytoplasmic mislocalization of RNA splicing factors and aberrant neuronal gene splicing in TDP-43 transgenic pig brain
... In TDP-43 transgenic pig brains, we saw diffuse stain- ing of TDP-43 in the cytoplasm without clear ...cytoplasmic TDP-43 aggregates sup- ports the idea that soluble ... See full document
20
Hepatitis B virus transactivator X protein is not tumorigenic in transgenic mice.
... Although the expression of mouse AAT protein in the transgenic mice remained high throughout the life span of the animals 43, the pattern of transgene expression in the ATX transgenic mi[r] ... See full document
9
TDP-43 Potentiates Alpha-synuclein Toxicity to Dopaminergic Neurons in Transgenic Mice
... of TDP-43 causes a defect at the neuromuscular junction in Drosophila melanogaster ...of TDP-43 in mice induces severe phenotypes (52, 53), underscor- ing the importance of ... See full document
10
Sustained Expression of TDP-43 and FUS in Motor Neurons in Rodent's Lifetime
... gene expression is sufficient to induce neu- ...(MPTP) induces selective loss of dopaminergic neu- rons in human and in animals [56, ...in transgenic mice [58], de- letion of the ... See full document
11
Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T)mice
... intrinsic neuronal origin. Immunostaining with a specific human TDP-43 antibody [22] confirmed the selective expression of the transgene in the nuclei of myenteric neurons (and glial cells), ... See full document
14
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
... protein 43 (TDP-43), a nuclear DNA/RNA-binding protein involved in RNA metabolism, is a pathological hallmark of amyotrophic lateral sclerosis ...prone, TDP-43 C-terminal domain is ... See full document
15
Amyotrophic lateral sclerosis mutant TDP 43 may cause synaptic dysfunction through altered dendritic spine function
... or mutant TDP-43 is suspected to play a major role in ALS pathogenesis (Buratti and Baralle, 2008; Sreedharan et ...how TDP-43 dysfunction or mutation is linked to altered ... See full document
11
Hsp90 and its Co-chaperones Modify TDP-43 Localization, Aggregation, and Toxicity
... protein 43 (TDP-43), is a 43 kDa DNA/RNA-binding protein of 414 amino acids encoded by the TAR DNA-binding protein (TARDBP) gene located on chromosome ...[67]. TDP-43 contains a ... See full document
113
RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy
... mediating TDP-43/RNA interactions whereas RRM2 has a lower affinity for RNA and has a predomin- antly regulatory function (Buratti and Baralle, 2001; Lukavsky et ...of TDP-43 to its target RNA, ... See full document
19
A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)
... upper edge of the granule cell layer. The data were then expressed per 250 x 50m sample field so that they were directly comparable with those from the other brain regions. The NCI [34,35, GI [36-41], NII [42] and DN ... See full document
37
Mutant TDP 43 in motor neurons promotes the onset and progression of ALS in rats
... protein 43 (TDP-43) has been linked to the development of an inherited form of ...Existing TDP-43 transgenic animals develop a limited loss of motor neurons and therefore do not ... See full document
13
Effects of mutant TDP-43 on the Nrf2/ARE pathway and protein expression of MafK and JDP2 in NSC-34 cells.
... Amyotrophic lateral sclerosis (ALS) is a motor neuron disorder that manifests as a chronic, progressive, and fatal neurodegenerative disease (Zarei et al., 2015). At present, the disease pathogenesis has not been ... See full document
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