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Showing 1 to 7 of 7 for “"Hutchinson-Gilford progeria syndrome"”.

  1. Mechano-sensitivity of nuclear lamin proteins in endothelial cells

    … studies have showed many similarities between Hutchinson Gilford Progeria Syndrome (HGPS) cells and aging cells, implicating dysfunctions of lamin A/C in aging process and atherosclerosis, as HGPS is caused by a mutated form of lamin A/C. Blood flow in arteries is generating shear stress that …

    iupui Repository record for Mechano-sensitivity of nuclear lamin proteins in endothelial cells (opens in a new tab)

  2. Neuronal regulation of haematopoietic stem cell ageing and age-related blood disorders through the microenvironment

    … premature haematopoietic ageing is observed in Hutchinson-Gilford progeria syndrome (HGPS). Chronic treatment of β3-AR agonist partially rejuvenates premature haematopoietic ageing in HGPS and restores exacerbated megakaryopoiesis in myeloproliferative neoplasms (MPNs). In summary, these results …

    cambridge Repository record for Neuronal regulation of haematopoietic stem cell ageing and age-related blood disorders through the microenvironment (opens in a new tab)

  3. 1, Structural and Functional Studies of Human Replication Protein A; 2 DNA Damage Responses and DNA Repair Defects in Laminopathy-Based Premature Aging.

    … been widely implicated in premature aging. In Hutchinson-Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD), premature aging is caused by defective maturation of lamin A and linked to accumulation of DNA double-strand breaks (DSBs). However, how lamin A dysfunction leads to genome …

    etsu Repository record for 1, Structural and Functional Studies of Human Replication Protein A; 2 DNA Damage Responses and DNA Repair Defects in Laminopathy-Based Premature Aging. (opens in a new tab)

  4. The Convergence of Accelerated and Physiological Vascular Ageing: A Multi-Omic Analysis of 2D and 3D in vitro models of Progeria

    … suffering from the accelerated ageing disease, Hutchinson Gilford Progeria Syndrome (HGPS), provide an indefinite source of human cells which recapitulate several hallmarks of ageing. Furthermore, the cardiovascular pathology of HGPS closely resembles that of elderly individuals, with patients …

    cambridge Repository record for The Convergence of Accelerated and Physiological Vascular Ageing: A Multi-Omic Analysis of 2D and 3D in vitro models of Progeria (opens in a new tab)

  5. Structural and mechanical properties of intermediate filaments under extreme conditions and disease

    … to investigate the molecular mechanism of Hutchinson-Gilford progeria syndrome, a premature aging disease. We find that the mutated domain tail domain is more compact and stable than the normal one. This altered structure and stability may enhance the association of intermediate filaments …

    mit Repository record for Structural and mechanical properties of intermediate filaments under extreme conditions and disease (opens in a new tab)

  6. Novel functions of N-acetyltransferase 10 (NAT10) in DNA repair and replication with potential implications for premature ageing syndromes

    Hutchinson-Gilford Progeria Syndrome (HGPS) is an invariably fatal disease with a range of diverse symptoms that are normally associated with those of advanced age. In recent decades, clinicians and scientists together have made great progress in deriving the mechanisms of disease initiation and …

    cambridge Repository record for Novel functions of N-acetyltransferase 10 (NAT10) in DNA repair and replication with potential implications for premature ageing syndromes (opens in a new tab)