Speeding up the clock: The past, present and future of progeria (Q38675558)

5 September 2017

title

Speeding up the clock: The past, present and future of progeria (English)

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5 September 2017

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Vijay Swahari

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5 September 2017

Ayumi Nakamura

series ordinal

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5 September 2017

publication date

21 December 2015

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Development, Growth and Differentiation

5 September 2017

published in

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5 September 2017

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5 September 2017

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5 September 2017

page(s)

116-130

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Zinc metalloproteinase, ZMPSTE24, is mutated in mandibuloacral dysplasia

5 September 2017

cites work

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Do lamin A and lamin C have unique roles?

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Autophagy and amino acid homeostasis are required for chronological longevity in Saccharomyces cerevisiae

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RecQ helicases: suppressors of tumorigenesis and premature aging.

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p21 both attenuates and drives senescence and aging in BubR1 progeroid mice

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BubR1 insufficiency causes early onset of aging-associated phenotypes and infertility in mice

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Clearance of p16Ink4a-positive senescent cells delays ageing-associated disorders

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Increased expression of BubR1 protects against aneuploidy and cancer and extends healthy lifespan.

21 January 2018

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Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect.

21 January 2018

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Identification of a novel X-linked gene responsible for Emery-Dreifuss muscular dystrophy

21 January 2018

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Mechanisms of life span extension by rapamycin in the fruit fly Drosophila melanogaster

21 January 2018

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Once-yearly zoledronic acid for treatment of postmenopausal osteoporosis

21 January 2018

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Modulation of Ras and a-factor function by carboxyl-terminal proteolysis

21 January 2018

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Life at the edge: the nuclear envelope and human disease

21 January 2018

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The laminopathies: the functional architecture of the nucleus and its contribution to disease

21 January 2018

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Progeria syndromes and ageing: what is the connection?

21 January 2018

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Aging, cellular senescence, and cancer

21 January 2018

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Nuclear lamin A/C R482Q mutation in canadian kindreds with Dunnigan-type familial partial lipodystrophy

21 January 2018

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Rapamycin Reverses Cellular Phenotypes and Enhances Mutant Protein Clearance in Hutchinson-Gilford Progeria Syndrome Cells

21 January 2018

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Human laminopathies: nuclei gone genetically awry

21 January 2018

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Human RecQ helicases in DNA repair, recombination, and replication.

21 January 2018

1 reference

The posttranslational processing of prelamin A and disease.

21 January 2018

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An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria

21 January 2018

1 reference

Lamin a truncation in Hutchinson-Gilford progeria.

21 January 2018

1 reference

The Hutchinson-Gilford progeria syndrome. Report of 4 cases and review of the literature

21 January 2018

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Nuclear lamins: major factors in the structural organization and function of the nucleus and chromatin

21 January 2018

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Telomere length in Hutchinson-Gilford progeria syndrome

21 January 2018

1 reference

The role of senescent cells in ageing

21 January 2018

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A Review of its Symptomatology, Natural History, Pathologic Features, Genetics And Relationship to the Natural Aging Process

21 January 2018

1 reference

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

21 January 2018

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Shorter telomeres, accelerated ageing and increased lymphoma in DNA-PKcs-deficient mice

21 January 2018

1 reference

Missense mutations in the rod domain of the lamin A/C gene as causes of dilated cardiomyopathy and conduction-system disease

21 January 2018

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

21 January 2018

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Prelamin A and lamin A appear to be dispensable in the nuclear lamina

21 January 2018

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Identification and chromosomal location of two human genes encoding enzymes potentially involved in proteolytic maturation of farnesylated proteins

21 January 2018

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Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts

21 January 2018

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

21 January 2018

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

21 January 2018

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Disease progression in Hutchinson-Gilford progeria syndrome: impact on growth and development

21 January 2018

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

21 January 2018

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Impact of farnesylation inhibitors on survival in Hutchinson-Gilford progeria syndrome.

21 January 2018

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Is isoprenylcysteine carboxyl methyltransferase the key to reverse ageing?

21 January 2018

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Rapamycin activates autophagy in Hutchinson-Gilford progeria syndrome: implications for normal aging and age-dependent neurodegenerative disorders

21 January 2018

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Prevention of Cardiovascular Events and Death with Pravastatin in Patients with Coronary Heart Disease and a Broad Range of Initial Cholesterol Levels

21 January 2018

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The nuclear lamina comes of age.

21 January 2018

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Lamins A and C are not expressed at early stages of human lymphocyte differentiation

21 January 2018

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A role for autophagy in the extension of lifespan by dietary restriction in C. elegans

21 January 2018

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THE LIMITED IN VITRO LIFETIME OF HUMAN DIPLOID CELL STRAINS

21 January 2018

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The serial cultivation of human diploid cell strains

21 January 2018

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Hutchinson-Gilford progeria syndrome: review of the phenotype.

21 January 2018

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Cellular senescence in aging primates.

21 January 2018

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The spectrum of WRN mutations in Werner syndrome patients

21 January 2018

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Accelerated telomere shortening and replicative senescence in human fibroblasts overexpressing mutant and wild-type lamin A.

21 January 2018

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Lamins: building blocks or regulators of gene expression?

21 January 2018

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A-type lamins: guardians of the soma?

21 January 2018

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

21 January 2018

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New approaches to progeria

21 January 2018

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21 January 2018

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Ink4a/Arf expression is a biomarker of aging

21 January 2018

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Werner and Hutchinson-Gilford progeria syndromes: mechanistic basis of human progeroid diseases.

21 January 2018

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Differential expression of nuclear lamin proteins during chicken development

21 January 2018

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Genomic instability in laminopathy-based premature aging

21 January 2018

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Recapitulation of premature ageing with iPSCs from Hutchinson-Gilford progeria syndrome

21 January 2018

1 reference

Zoledronic acid and clinical fractures and mortality after hip fracture

21 January 2018

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

21 January 2018

1 reference

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

21 January 2018

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What geriatricians should know about the Werner syndrome

21 January 2018

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Vitamin C restores healthy aging in a mouse model for Werner syndrome

21 January 2018

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Induction of nuclear lamins A/C during in vitro-induced differentiation of F9 and P19 embryonal carcinoma cells

21 January 2018

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Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial

21 January 2018

1 reference

Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody

21 January 2018

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

21 January 2018

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Phenotype and course of Hutchinson-Gilford progeria syndrome

21 January 2018

1 reference

Rapamycin, but not resveratrol or simvastatin, extends life span of genetically heterogeneous mice

21 January 2018

1 reference

Pharmacological inhibition of phosphoinositide 3 and TOR kinases improves survival of Drosophila melanogaster.

21 January 2018

1 reference

Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy

21 January 2018

1 reference

A progeroid syndrome in mice is caused by defects in A-type lamins

21 January 2018

1 reference

Cockayne syndrome: Review of 140 cases

21 January 2018

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Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear disorganization and identify restrictive dermopathy as a lethal neonatal laminopathy

21 January 2018

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Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of Lamin A precursors.

21 January 2018

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

21 January 2018

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Proteomic profiling of adipose tissue from Zmpste24-/- mice, a model of lipodystrophy and premature aging, reveals major changes in mitochondrial function and vimentin processing

21 January 2018

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Progeria: A cell culture study and clinical report of familial incidence

21 January 2018

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Inhibition of purified p21ras farnesyl:protein transferase by Cys-AAX tetrapeptides

21 January 2018

1 reference

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

21 January 2018

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Initial cutaneous manifestations of Hutchinson-Gilford progeria syndrome

21 January 2018

1 reference

Werner's syndrome: a review of recent research with an analysis of connective tissue metabolism, growth control of cultured cells, and chromosomal aberrations

21 January 2018

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Lamin A-dependent nuclear defects in human aging

21 January 2018

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Senescing human cells and ageing mice accumulate DNA lesions with unrepairable double-strand breaks

21 January 2018

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Compound heterozygous ZMPSTE24 mutations reduce prelamin A processing and result in a severe progeroid phenotype

21 January 2018

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A case of Werner syndrome with three primary lesions of malignant melanoma

21 January 2018

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Restoring systemic GDF11 levels reverses age-related dysfunction in mouse skeletal muscle

21 January 2018

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Smooth muscle cell depletion and collagen types in progeric arteries

21 January 2018

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Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy

21 January 2018

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

21 January 2018

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Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome

21 January 2018

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Puzzles, promises and a cure for ageing

21 January 2018

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A second higher vertebrate B-type lamin. cDNA sequence determination and in vitro processing of chicken lamin B2.

21 January 2018

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Transgenic studies with a keratin promoter-driven growth hormone transgene: prospects for gene therapy

21 January 2018

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Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin

21 January 2018

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Blocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndrome

21 January 2018

1 reference

An unidentified neonatal progeroid syndrome: follow-up report

21 January 2018

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Diseases of the nuclear envelope

21 January 2018

1 reference

A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation.

21 January 2018

1 reference

Metabolic improvement and abdominal fat redistribution in Werner syndrome by pioglitazone

21 January 2018

1 reference

Positional cloning of the Werner's syndrome gene

21 January 2018

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

21 January 2018

1 reference

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

21 January 2018

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

21 January 2018

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

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/DGD.12251

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5 September 2017

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