p300 nucleocytoplasmic shuttling underlies mTORC1 hyperactivation in Hutchinson-Gilford progeria syndrome. [PDF]
Son SM +4 more
europepmc +1 more source
The anti-senescence effect of D-β-hydroxybutyrate in Hutchinson-Gilford progeria syndrome involves progerin clearance by the activation of the AMPK-mTOR-autophagy pathway. [PDF]
Monterrubio-Ledezma F +5 more
europepmc +1 more source
Long lifetime and tissue-specific accumulation of lamin A/C in Hutchinson-Gilford progeria syndrome. [PDF]
Hasper J +5 more
europepmc +1 more source
The Use of Peripheral Nerve Block and Intra-articular Steroid Injection for Pain Management in an Adolescent with Hutchinson-Gilford Progeria Syndrome: A Case Report. [PDF]
Endencia MCS, Sy Su KMC, Reyes CRB.
europepmc +1 more source
Aging of Hutchinson–Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis [PDF]
Hutchinson-Gilford progeria syndrome is a rare genetic disorder that mimics certain aspects of aging prematurely. Recent work has revealed that mutations in the lamin A gene are a cause of the disease.
Joanna M Bridger, Ian R Kill
exaly +7 more sources
Anesthetic considerations in children with Hutchinson-Gilford progeria syndrome: A narrative review
BACKGROUND: Hutchinson-Gilford progeria syndrome is a rare disease in childhood that results in premature aging. The presence of multisystem derangements including skin, bone, and joint diseases and possibly a difficult airway makes the anesthetic ...
Kristof Nijs, Marc van de Velde
exaly +2 more sources
Mechanisms of Premature Vascular Aging in Children With Hutchinson-Gilford Progeria Syndrome
Hutchinson-Gilford progeria syndrome is a rare, segmental premature aging syndrome of accelerated atherosclerosis and early death from myocardial infarction or stroke.
Armin Schwartzman +2 more
exaly +2 more sources
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Hutchinson–Gilford progeria syndrome
Clinical Genetics, 2004Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder that causes premature, rapid aging shortly after birth. Recently, de novo point mutations in the Lmna gene have been found in individuals with HGPS. Lmna encodes lamin A and C, the A‐type lamins, which are an important structural component of the nuclear envelope.
R L, Pollex, R A, Hegele
openaire +3 more sources
Telomere length in Hutchinson-Gilford Progeria Syndrome
Mechanisms of Ageing and Development, 2009Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare premature aging disorder caused by mutations in the gene LMNA, which encodes the nuclear matrix protein lamin A. Previous research has shown that the average telomere length in fibroblasts from HGPS patients is shorter than in age-matched controls. How mutations in lamin A lead to shortened telomere
Peter Lansdorp
exaly +3 more sources

