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Biology Open, 2012 Summary Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by segmental accelerated aging and early death from coronary artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation within exon 11 of
Vera Wenzel +7 more
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An upregulation in the expression of vanilloid transient potential channels 2 enhances hypotonicity-induced cytosolic Ca²⁺ rise in human induced pluripotent stem cell model of Hutchinson-Gillford Progeria. [PDF]
PLoS ONE, 2014 Hutchinson-Gillford Progeria Syndrome (HGPS) is a fatal genetic disorder characterized by premature aging in multiple organs including the skin, musculoskeletal and cardiovascular systems. It is believed that an increased mechanosensitivity of HGPS cells
Chun-Yin Lo +8 more
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Frontiers in Physiology, 2022 Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder that causes accelerated aging and a high risk of cardiovascular complications. However, the underlying mechanisms of cardiac complications of this syndrome are not fully understood ...
Gustavo Monnerat +17 more
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The Impact of Nutrient Restriction and Metformin Treatment on Genome Structure and Function in Hutchinson-Gilford Progeria Syndrome (HGPS) Fibroblasts [PDF]
, 2022 Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare disease in which children age at an accelerated rate. Patients suffering from HGPS have a shortened lifespan and succumb to cardiac events at ~14 years of age.
Gillespie, Zoe E
core
Loss of H3K9me3 Correlates with ATM Activation and Histone H2AX Phosphorylation Deficiencies in Hutchinson-Gilford Progeria Syndrome. [PDF]
PLoS ONE, 2016 Compelling evidence suggests that defective DNA damage response (DDR) plays a key role in the premature aging phenotypes in Hutchinson-Gilford progeria syndrome (HGPS).
Haoyue Zhang +6 more
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HGPS (Hutchinson-Gilford -Progeria syndrome)
IP Journal of Surgery and Allied Sciences, 2020 Eight year old male child, born of non- consanguinity presented with complaints of irritability and drowsiness with history of left sided weakness. He was stunted and had hypertension at presentation. Also he had dysmorphic features and skin manifestations with skeletal deformities and muscle wasting.
Santosh Kondekar +4 more
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Nucleus, 2019 Lamin A, a product of the LMNA gene, is an essential nuclear envelope component in most differentiated cells. Mutations in LMNA have been linked to premature aging disorders, including Hutchinson-Gilford progeria syndrome (HGPS).
Magda R. Hamczyk, Vicente Andrés
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Structure and stability of the lamin A tail domain and HGPS mutant [PDF]
Journal of Structural Biology, 2011 Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging syndrome caused by the expression and accumulation of a mutant form of lamin A, Δ50 lamin A. As a component of the cell's nucleoskeleton, lamin A plays an important role in the mechanical stabilization of the nuclear envelope and in other nuclear functions.
Qin, Zhao +3 more
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IEEE Access, 2023 Multi-vector smart local energy systems are playing an increasingly importantly role in the fast-track decarbonisation of our global energy services. An emergent contributor to global decarbonisation is green hydrogen. Green hydrogen can remove or reduce
Hani Gharavi Ahangar +2 more
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Loss of H3K9me3 correlates with impaired ATM activation in HGPS.
, 2016 (A). Representative fluorescence images of gammaH2AX and H3K9me3 in middle passage normal and HGPS fibroblasts after Dox treatment. “Low H3K9me3” is defined as any H3K9me3 fluorescence intensity that falls below the lower 5% of the normal population ...
Celeste Witting (3372776) +6 more
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