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Complete suppression of Htt fibrilization and ...

2017-12-6 · A dynamic chaperone complex of Hsc70, Hsp110 and J-protein dissolves pathological Huntingtin fibrils in vitro and prevents aggregate formation in Huntington''s disease patient-derived neurons.. Hsc70, a type B J-protein and Hsp110 fully supress fibrilization of huntingtin exon1 for at least 24 h in vitro.; The same chaperones can disaggregate preformed Huntingtin (Htt) fibrils.

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Frontiers | Cellular Regulation of Amyloid Formation in ...

Loss of NPC proteins were found to enhance G 4 C 2 repeat toxicity in fly and human cell models for disease (Freibaum et al., 2015; Zhang et al., 2015). Moreover, a screen to identify modifiers of toxicity by PR 50 DPR identified an enrichment in …

Adjunct Members | Institute Of Infectious Disease and ...

2021-9-5 · Adjunct membership is for researchers employed by other institutions who collaborate with IDM Members to the extent that some of their own staff and/or postgraduate students may work within the IDM; for 3-year terms, which are renewable.

Formation of adenovirus DNA replication compartments ...

2019-11-11 · Techniques for the study of AdRCs: advantages and shortcomings. Adenovirus replication compartments have been studied by fluorescence, super-resolution, and electron microscopy in infected cells and by biochemical techniques that employ drugs or mutant viruses that encode temperature-sensitive proteins or proteins that bear specific residue substitutions and that are known (or …

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Chromatin Evolution and Molecular Drive in Speciation

The NPC forms channels that allow transport of macromolecules between the nucleus and cytoplasm (for a recent review see ). In addition, NPC components also function in kinetochore/spindle formation and transcriptional regulation (i.e., dosage compensation) [86–91]. The evolution of scaffold nucleoporins (the NUP107-160 complex) may have ...

Cytoplasm: The Secretory System | Basicmedical Key

2017-7-9 · and Jürgen Roth2 (1) Medical University of Vienna, Vienna, Austria (2) University of Zurich, Zurich, Switzerland Secretory Pathway of Pancreatic Acinar Cells In their classical Nobel Prize–winning studies on protein biosynthesis and secretion, Palade and coworkers used the exocrine pancreas as a model tissue. The low-power electron micrograph from rat exocrine pancreas shows acinar cells…

Pervasive Adaptive Evolution among Interactors of the ...

Lineage-Specific Adaptive Evolution. To determine whether the adaptive evolution of any particular nucleoporin gene was restricted to the D. melanogaster lineage or to the D. simulans lineage, or occurred in both lineages, we performed lineage-specific MK tests. Table 2 shows the results of 3 of these tests for each gene. For the first test, D. simulans and D. yakuba sequences were used as ...

Small-molecule G-quadruplex stabilizers reveal a novel ...

2020-2-11 · (a) A scheme of the rat Atg7 gene and its promoter showing putative G4-DNA locations. (b–d) Cultured primary neurons were treated with a vehicle (control, cont) or with PDS (2 μM) overnight.Neurons were collected and processed to measure mRNA …

NUCLEAR ASSEMBLY | Annual Review of Cell and …

2019-7-17 · This suggests that NPC structure helps control the morphology of the nuclear membrane where it bends to encircle the NPC. There is a mutant in S. cerevisiae defective in the synthesis of very-long chain (C26) fatty acids, including sphingolipids; at the nonpermissive temperature, the outer and inner membranes become widely separated, and NPC ...

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Integrated Genomic Analysis Suggests MLL3 Is a Novel ...

Background: Little is known about genetic factors associated with nasopharyngeal carcinoma (NPC). To gain insight into NPC etiology, we performed whole exome sequencing on germline and tumor DNA from three closely related family members with NPC. Methods: The family was ascertained through the Pediatric Familial Cancer Clinic at The University of Chicago (Chicago, IL). The diagnosis of NPC was ...

Genetic diversity and complexity of Plasmodium …

2014-5-1 · Results. All the families of msp-1 (K1, MAD20 and R033) and msp-2 (FC27 and 3D7) locus were observed.Results revealed that K1 (60/100) was the most predominant genotype of msp-1 allelic family followed by the genotypes of MAD20 (50/100) and R033 (45/100) the msp-2 locus, FC27 genotype (62/100) showed higher frequency than 3D7 genotype (55/100).The allelic families were …

Dysregulated molecular pathways in amyotrophic lateral ...

2017-9-15 · Frontotemporal dementia (FTD), the second most common form of dementia in people under 65 years of age, is characterized by progressive atrophy of the frontal and/or temporal lobes.FTD overlaps extensively with the motor neuron disease amyotrophic lateral sclerosis (ALS), especially at the genetic level.Both FTD and ALS can be caused by many mutations in the same set of genes; the most ...

Lineage-Specific Evolution of the Complex Nup160 Hybrid ...

2015-8-1 · Two genes encoding protein components of the nuclear pore complex [Nup160][1] and Nup96 cause lethality in F2-like hybrid genotypes between Drosophila simulans and Drosophila melanogaster . In particular, D. simulans Nup160 and Nup96 each cause inviability when hemizygous or homozygous in species hybrids that are also hemizygous (or homozygous) for the D. melanogaster X chromosome. …

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Membrane Trafficking Pathways in Alzheimer''s Disease ...

2012-1-23 · Alzheimer''s disease (AD) is the most common dementia, characterized by the presence of amyloid plaques and neurofibrillary tangles 1.Tangles consist of intracellular hyperphosphorylated tau 2, 3, while amyloid plaques are extracellular and mainly composed of amyloid β (Aβ) peptides 4.All players involved in the generation of Aβ, including the substrate amyloid precursor protein (APP) and α ...

Increased exosome secretion in neurons aging in vitro by ...

2021-6-28 · As neurons age, they show a decrease in their ability to degrade proteins and membranes. Because undegraded material is a source of toxic products, defects in degradation are associated with reduced cell function and survival. However, there are very few dead neurons in the aging brain, suggesting the action of compensatory mechanisms. We show in this work that ageing neurons in …

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Poor old pores-The challenge of making and maintaining ...

2020-1-23 · The nuclear pore complex (NPC) is the sole gateway to the nuclear interior, and its function is essential to all eukaryotic life ntrolling the functionality of NPC s is a tremendous challenge for cells. Firstly, NPC s are large structures, and their complex assembly does occasionally go awry. Secondly, once assembled, some components of the NPC persist for an extremely long time and, as a ...

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Integrated Genomic Analysis Suggests MLL3 Is a Novel ...

Background: Little is known about genetic factors associated with nasopharyngeal carcinoma (NPC). To gain insight into NPC etiology, we performed whole exome sequencing on germline and tumor DNA from three closely related family members with NPC. Methods: The family was ascertained through the Pediatric Familial Cancer Clinic at The University of Chicago (Chicago, IL).

Genome instability and loss of protein homeostasis ...

2021-4-21 · Genome instability and loss of protein homeostasis are hallmark events of age-related diseases that include neurodegeneration. Several neurodegenerative diseases, such as Alzheimer''s disease, Parkinson''s disease, Huntington''s disease and amyotrophic lateral sclerosis are characterized by protein aggregation, while an impaired DNA damage response (DDR) as in many genetic DNA …