Progeria: a paradigm for translational medicine. (Q47917390)

http://europepmc.org/abstract/MED/24485450

6 February 2018

title

Progeria: a paradigm for translational medicine (English)

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6318797&resulttype=core&format=json

13 May 2020

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3

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4

Tom Misteli

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1

Leslie B Gordon

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6318797&resulttype=core&format=json

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author name string

Frank G Rothman

series ordinal

2

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13 May 2020

language of work or name

English

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publication date

1 January 2014

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http://europepmc.org/abstract/MED/24485450

6 February 2018

published in

Cell

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6318797&resulttype=core&format=json

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volume

156

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issue

3

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page(s)

400-407

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Lamin a truncation in Hutchinson-Gilford progeria.

13 May 2020

cites work

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12 December 2020

Recurrent de novo point mutations in lamin A cause Hutchinson–Gilford progeria syndrome

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12 December 2020

Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome

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12 December 2020

Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome

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12 December 2020

Reduced adiponectin and HDL cholesterol without elevated C-reactive protein: clues to the biology of premature atherosclerosis in Hutchinson-Gilford Progeria Syndrome

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12 December 2020

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.

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12 December 2020

Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation.

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12 December 2020

Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition

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12 December 2020

Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome

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12 December 2020

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

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12 December 2020

Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome

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12 December 2020

Correction of cellular phenotypes of Hutchinson-Gilford Progeria cells by RNA interference

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12 December 2020

A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria.

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12 December 2020

Lamin A-dependent nuclear defects in human aging

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12 December 2020

DNA damage responses in progeroid syndromes arise from defective maturation of prelamin A.

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12 December 2020

Progeria of stem cells: stem cell exhaustion in Hutchinson-Gilford progeria syndrome

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12 December 2020

"Laminopathies": a wide spectrum of human diseases

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12 December 2020

New approaches to progeria

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12 December 2020

The mutant form of lamin A that causes Hutchinson-Gilford progeria is a biomarker of cellular aging in human skin

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12 December 2020

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation.

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12 December 2020

Phenotype and course of Hutchinson-Gilford progeria syndrome

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12 December 2020

Lamin A-dependent misregulation of adult stem cells associated with accelerated ageing

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12 December 2020

Nuclear envelope defects cause stem cell dysfunction in premature-aging mice

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12 December 2020

Premature aging in mice activates a systemic metabolic response involving autophagy induction.

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12 December 2020

Combined treatment with statins and aminobisphosphonates extends longevity in a mouse model of human premature aging.

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12 December 2020

A farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse model

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12 December 2020

Suppression of proliferative defects associated with processing-defective lamin A mutants by hTERT or inactivation of p53.

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12 December 2020

Increased expression of the Hutchinson-Gilford progeria syndrome truncated lamin A transcript during cell aging

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12 December 2020

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12 December 2020

Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A.

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12 December 2020

Mechanobiology and the microcirculation: cellular, nuclear and fluid mechanics

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12 December 2020

Prelamin A acts to accelerate smooth muscle cell senescence and is a novel biomarker of human vascular aging

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12 December 2020

Role of progerin-induced telomere dysfunction in HGPS premature cellular senescence

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12 December 2020

Progeria syndromes and ageing: what is the connection?

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12 December 2020

Cardiovascular pathology in Hutchinson-Gilford progeria: correlation with the vascular pathology of aging.

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12 December 2020

Insulin-like growth factor 1 treatment extends longevity in a mouse model of human premature aging by restoring somatotroph axis function.

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12 December 2020

Congenital Absence of Hair and Mammary Glands with Atrophic Condition of the Skin and its Appendages, in a Boy whose Mother had been almost wholly Bald from Alopecia Areata from the age of Six.

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12 December 2020

A human iPSC model of Hutchinson Gilford Progeria reveals vascular smooth muscle and mesenchymal stem cell defects

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12 December 2020

The role of cellular senescence during vascular calcification: a key paradigm in aging research.

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12 December 2020

Hutchinson-Gilford progeria is a skeletal dysplasia

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12 December 2020

Aging and chronic DNA damage response activate a regulatory pathway involving miR-29 and p53.

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12 December 2020

The vascular adventitia: its role in the arterial injury response

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12 December 2020

Targeted gene correction of laminopathy-associated LMNA mutations in patient-specific iPSCs

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12 December 2020

Progerin and telomere dysfunction collaborate to trigger cellular senescence in normal human fibroblasts

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12 December 2020

Challenging issues in pediatric oncology

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12 December 2020

Rapamycin reverses cellular phenotypes and enhances mutant protein clearance in Hutchinson-Gilford progeria syndrome cells

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12 December 2020

Stem cell depletion in Hutchinson-Gilford progeria syndrome

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12 December 2020

Splicing-Directed Therapy in a New Mouse Model of Human Accelerated Aging

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12 December 2020

Coronary artery disease in a Werner syndrome-like form of progeria characterized by low levels of progerin, a splice variant of lamin A.

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12 December 2020

Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome

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12 December 2020

RNA therapeutics: beyond RNA interference and antisense oligonucleotides

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12 December 2020

Autophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria.

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12 December 2020

Regulation of prelamin A but not lamin C by miR-9, a brain-specific microRNA.

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12 December 2020

A prospective study of radiographic manifestations in Hutchinson-Gilford progeria syndrome

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12 December 2020

Temsirolimus activates autophagy and ameliorates cardiomyopathy caused by lamin A/C gene mutation

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12 December 2020

Rapamycin reverses elevated mTORC1 signaling in lamin A/C-deficient mice, rescues cardiac and skeletal muscle function, and extends survival.

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12 December 2020

Unique Preservation of Neural Cells in Hutchinson- Gilford Progeria Syndrome Is Due to the Expression of the Neural-Specific miR-9 MicroRNA

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12 December 2020

Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndrome

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12 December 2020

Nuclear lamina defects cause ATM-dependent NF-κB activation and link accelerated aging to a systemic inflammatory response

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12 December 2020

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12 December 2020

Imaging characteristics of cerebrovascular arteriopathy and stroke in Hutchinson-Gilford progeria syndrome

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12 December 2020

The nuclear lamins: flexibility in function

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12 December 2020

Naïve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo

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12 December 2020

Resveratrol rescues SIRT1-dependent adult stem cell decline and alleviates progeroid features in laminopathy-based progeria

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12 December 2020

Prelamin A accelerates vascular calcification via activation of the DNA damage response and senescence-associated secretory phenotype in vascular smooth muscle cells.

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12 December 2020

Targeting isoprenylcysteine methylation ameliorates disease in a mouse model of progeria.

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12 December 2020

The hallmarks of aging

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12 December 2020

Moving from gene discovery to clinical trials in Hutchinson-Gilford progeria syndrome

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12 December 2020

Neurologic features of Hutchinson-Gilford progeria syndrome after lonafarnib treatment

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12 December 2020

Prelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion.

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12 December 2020

Cardiovascular abnormalities in progeria. Case report and review of the literature.

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12 December 2020

The processing pathway of prelamin A

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12 December 2020

10.1016/J.CELL.2013.12.028

Identifiers

DOI

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6318797&resulttype=core&format=json

13 May 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6318797&resulttype=core&format=json

13 May 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=PMCID:PMC6318797&resulttype=core&format=json