Progeria syndrome

Disease

2 papers study it383 more mention it1 finding

Humans1

Effects

Pathological changes of normal aging

upin humans1

1 study

Progeria syndrome increases pathological changes of normal aging in humans.
“progeria syndromes accelerate a subset of the pathological changes that together drive the normal ageing process”
Review
Progeria syndromes and ageing: what is the connection?
Nature reviews. Molecular cell biologyAug 1, 2010

Funding

13 grants since 2016

National Institute on AgingUS$4.5mNational Heart, Lung, and Blood InstituteUS$3.1mJapan Society for the Promotion of ScienceJP¥9.4m

Latest

46 papers in 12 months

Aging CellOct 15

Restoring a protein partly improves heart function in mice

Experiments in mice producing a progeria-linked protein in heart muscle cells found that restoring an RNA-marking protein partly reduced abnormal heart changes.Experiments in mice producing a progeria-linked protein in heart muscle cells found that restoring an RNA-marking protein partly reduced abnormal heart changes.

Mouse studyMechanisms

Paper

bioRxivOct 14

Progeria disrupts cells in the intestinal lining in mice

Mice carrying one copy of a progeria-linked mutation developed increased gut leakage from 30 weeks, reaching levels reported in naturally aged mice over 20 months old.Mice carrying one copy of a progeria-linked mutation developed increased gut leakage from 30 weeks, reaching levels reported in naturally aged mice over 20 months old.

Preprint: mouse studyLMNA

Paper

Science AdvancesOct 7

Support cells worsened disease features in lab-grown human vessels

Experiments with three-layer vessels built from human cells found that support cells increased several vascular disease features in a model of Hutchinson-Gilford progeria…Experiments with three-layer vessels built from human cells found that support cells increased several vascular disease features in a model of Hutchinson-Gilford progeria syndrome.

Experiments in human cellsMechanisms

Paper

NPJ AgingOct 7

Human protein variant eased signs of progeria in mice

An animal experiment in genetically engineered mice found that the variant prevented characteristic tissue changes and modestly increased median lifespan.An animal experiment in genetically engineered mice found that the variant prevented characteristic tissue changes and modestly increased median lifespan.

Experiments in miceInterventions

Paper

Nature CommunicationsOct 1

Base editing of the lamin farnesylation motif rescues progeria phenotypes in organoids

Lipid nanoparticle delivery of base-editing mRNA restored DNA repair and heterochromatin structure in human neuromuscular models of premature aging.Lipid nanoparticle delivery of base-editing mRNA restored DNA repair and heterochromatin structure in human neuromuscular models of premature aging.

Experiments in human cellsProgeria

Paper

Pharmacological ResearchSep 30

Extracellular vesicles carrying two microRNAs reduce vascular aging markers in progeria cells

Vesicles from melatonin-treated stem cells targeted SRSF1 and SP1 to lower progerin and cellular prion protein in cell models.Vesicles from melatonin-treated stem cells targeted SRSF1 and SP1 to lower progerin and cellular prion protein in cell models.

Experiments in human cellsProgeria

Paper

bioRxivSep 18