Search Results - "Mediani, Laura"
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A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone Complex Ensures Stress Granule Integrity and Dynamism
Published in Molecular cell (01-09-2016)“…Stress granules (SGs) are ribonucleoprotein complexes induced by stress. They sequester mRNAs and disassemble when the stress subsides, allowing translation…”
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BAG3 Pro209 mutants associated with myopathy and neuropathy relocate chaperones of the CASA-complex to aggresomes
Published in Scientific reports (29-05-2020)“…Three missense mutations targeting the same proline 209 (Pro209) codon in the co-chaperone Bcl2-associated athanogene 3 (BAG3) have been reported to cause…”
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3
Studying heat shock proteins through single-molecule mechanical manipulation
Published in Cell stress & chaperones (01-07-2020)“…Imbalances of cellular proteostasis are linked to ageing and human diseases, including neurodegenerative and neuromuscular diseases. Heat shock proteins (HSPs)…”
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HspB8 prevents aberrant phase transitions of FUS by chaperoning its folded RNA-binding domain
Published in eLife (06-09-2021)“…Aberrant liquid-to-solid phase transitions of biomolecular condensates have been linked to various neurodegenerative diseases. However, the underlying…”
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Small heat-shock protein HSPB3 promotes myogenesis by regulating the lamin B receptor
Published in Cell death & disease (06-05-2021)“…One of the critical events that regulates muscle cell differentiation is the replacement of the lamin B receptor (LBR)-tether with the lamin A/C (LMNA)-tether…”
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Alternatively spliced exon regulates context-dependent MEF2D higher-order assembly during myogenesis
Published in Nature communications (10-03-2023)“…During muscle cell differentiation, the alternatively spliced, acidic β-domain potentiates transcription of Myocyte-specific Enhancer Factor 2 (Mef2D)…”
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Protein products of nonstop mRNA disrupt nucleolar homeostasis
Published in Cell stress & chaperones (01-05-2021)“…Stalled mRNA translation results in the production of incompletely synthesized proteins that are targeted for degradation by ribosome-associated quality…”
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Aberrant Compartment Formation by HSPB2 Mislocalizes Lamin A and Compromises Nuclear Integrity and Function
Published in Cell reports (Cambridge) (29-08-2017)“…Small heat shock proteins (HSPBs) contain intrinsically disordered regions (IDRs), but the functions of these IDRs are still unknown. Here, we report that, in…”
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BAG3 and BAG6 differentially affect the dynamics of stress granules by targeting distinct subsets of defective polypeptides released from ribosomes
Published in Cell stress & chaperones (01-11-2020)“…Stress granules (SGs) are dynamic ribonucleoprotein granules induced by environmental stresses. They play an important role in the stress response by…”
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10
Heat Shock Proteins: Important Helpers for the Development, Maintenance and Regeneration of Skeletal Muscles
Published in Muscles (18-04-2023)“…The skeletal muscle is a highly plastic tissue that shows a remarkable adaptive capacity in response to acute and resistance exercise, and modifies its…”
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Loss of PML nuclear bodies in familial amyotrophic lateral sclerosis-frontotemporal dementia
Published in Cell death discovery (15-07-2023)“…Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) are two neurodegenerative disorders that share genetic causes and pathogenic mechanisms…”
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12
Reversal of the glycolytic phenotype of primary effusion lymphoma cells by combined targeting of cellular metabolism and PI3K/Akt/ mTOR signaling
Published in Oncotarget (02-02-2016)“…PEL is a B-cell non-Hodgkin lymphoma, occurring predominantly as a lymphomatous effusion in body cavities, characterized by aggressive clinical course, with no…”
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13
Targeting PI3K/AKT/mTOR network for treatment of leukemia
Published in Cellular and molecular life sciences : CMLS (01-06-2015)“…OBJECTIVE: Increased activity of PI3K/AKT/mTOR pathway has been observed in a huge number of malignancies. This pathway can function as a prosurvival factor in…”
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14
ALS and FTD: Where RNA metabolism meets protein quality control
Published in Seminars in cell & developmental biology (01-03-2020)“…Recent genetic and biochemical evidence has improved our understanding of the pathomechanisms that lead to amyotrophic lateral sclerosis (ALS) and…”
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15
Granulostasis: Protein Quality Control of RNP Granules
Published in Frontiers in molecular neuroscience (27-03-2017)“…Ribonucleoprotein (RNP) granules transport, store, or degrade messenger RNAs, thereby indirectly regulating protein synthesis. Normally, RNP granules are…”
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Defective ribosomal products challenge nuclear function by impairing nuclear condensate dynamics and immobilizing ubiquitin
Published in The EMBO journal (01-08-2019)“…Nuclear protein aggregation has been linked to genome instability and disease. The main source of aggregation‐prone proteins in cells is defective ribosomal…”
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Hsp90-mediated regulation of DYRK3 couples stress granule disassembly and growth via mTORC1 signaling
Published in EMBO reports (05-05-2021)“…Stress granules (SGs) are dynamic condensates associated with protein misfolding diseases. They sequester stalled mRNAs and signaling factors, such as the…”
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Human Small Heat Shock Protein B8 Inhibits Protein Aggregation without Affecting the Native Folding Process
Published in Journal of the American Chemical Society (19-07-2023)“…Small Heat Shock Proteins (sHSPs) are key components of our Protein Quality Control system and are thought to act as reservoirs that neutralize irreversible…”
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Missense mutation in ATXN2 gene (c.2860C > T) in an amyotrophic lateral sclerosis patient with aggressive disease phenotype
Published in Neurological sciences (01-10-2022)“…Background ALS symptoms have been previously described only in the context of ATXN2 CAG expansions, whereas missense mutations of the gene have never been…”
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20
The protein kinase Akt/PKB regulates both prelamin A degradation and Lmna gene expression
Published in The FASEB journal (01-06-2013)“…The serine/threonine kinase Akt/PKB is a major signaling hub integrating metabolic, survival, growth, and cell cycle regulatory signals. The definition of the…”
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