Nuclear lamins and laminopathies

LMNA cardiomyopathy: cell biology and genetics meet clinical medicine

(2011) J. T. Lu et al.   Disease Models & Mechanisms

High prevalence of laminopathies among patients with metabolic syndrome

(2011) Anne Dutour et al.   HUMAN MOLECULAR GENETICS

An absence of both lamin B1 and lamin B2 in keratinocytes has no effect on cell proliferation or the development of skin and hair

(2011) Shao H. Yang et al.   HUMAN MOLECULAR GENETICS

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

(2011) Kan Cao et al.   JOURNAL OF CLINICAL INVESTIGATION

Highlights of the 2007 Progeria Research Foundation Scientific Workshop: Progress in Translational Science

(2011) L. B. Gordon et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

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

(2011) Guang-Hui Liu et al.   NATURE

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

(2011) K. Cao et al.   Science Translational Medicine

Pharmacological inhibition of c-Jun N-terminal kinase signaling prevents cardiomyopathy caused by mutation in LMNA gene

(2010) Wei Wu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mitogen-Activated Protein Kinase Inhibitors Improve Heart Function and Prevent Fibrosis in Cardiomyopathy Caused by Mutation in Lamin A/C Gene

(2010) Wei Wu et al.   CIRCULATION

Functional Coupling between the Extracellular Matrix and Nuclear Lamina by Wnt Signaling in Progeria

(2010) Lidia Hernandez et al.   DEVELOPMENTAL CELL

Defective DSB repair correlates with abnormal nuclear morphology and is improved with FTI treatment in Hutchinson-Gilford progeria syndrome fibroblasts

(2010) Dan Constantinescu et al.   EXPERIMENTAL CELL RESEARCH

Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin

(2010) Shao H. Yang et al.   HUMAN MOLECULAR GENETICS

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

(2010) E. K. Benson et al.   JOURNAL OF CELL SCIENCE

Lamin A variants that cause striated muscle disease are defective in anchoring transmembrane actin-associated nuclear lines for nuclear movement

(2010) E. S. Folker et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Abnormal development of the cerebral cortex and cerebellum in the setting of lamin B2 deficiency

(2010) C. Coffinier et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Diseases of the Nuclear Envelope

(2010) H. J. Worman et al.   Cold Spring Harbor Perspectives in Biology

The Posttranslational Processing of Prelamin A and Disease

(2009) Brandon S.J. Davies et al.   Annual Review of Genomics and Human Genetics

The Nuclear Envelope as a Signaling Node in Development and Disease

(2009) William T. Dauer et al.   DEVELOPMENTAL CELL

Atypical Progeroid Syndrome due to Heterozygous MissenseLMNAMutations

(2009) Abhimanyu Garg et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Laminopathies and the long strange trip from basic cell biology to therapy

(2009) Howard J. Worman et al.   JOURNAL OF CLINICAL INVESTIGATION

Increased mechanosensitivity and nuclear stiffness in Hutchinson–Gilford progeria cells: effects of farnesyltransferase inhibitors

(2008) Valerie L. R. M. Verstraeten et al.   AGING CELL

De novoLMNAmutations cause a new form of congenital muscular dystrophy

(2008) Susana Quijano-Roy et al.   ANNALS OF NEUROLOGY

Dysfunctional Connections Between the Nucleus and the Actin and Microtubule Networks in Laminopathic Models

(2008) Christopher M. Hale et al.   BIOPHYSICAL JOURNAL

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

(2008) T. Dechat et al.   GENES & DEVELOPMENT

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

(2008) Y. Wang et al.   HUMAN MOLECULAR GENETICS

Inhibition of extracellular signal-regulated kinase signaling to prevent cardiomyopathy caused by mutation in the gene encoding A-type lamins

(2008) A. Muchir et al.   HUMAN MOLECULAR GENETICS

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

(2008) Jesús Espada et al.   JOURNAL OF CELL BIOLOGY

Severe Mandibuloacral Dysplasia-Associated Lipodystrophy and Progeria in a Young Girl with a Novel Homozygous Arg527CysLMNAMutation

(2008) Anil K. Agarwal et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated

(2008) Shao H. Yang et al.   JOURNAL OF CLINICAL INVESTIGATION

Suppression of Proliferative Defects Associated with Processing-defective Lamin A Mutants by hTERT or Inactivation of p53

(2008) Brian A. Kudlow et al.   MOLECULAR BIOLOGY OF THE CELL

Transcriptional repression mediated by repositioning of genes to the nuclear lamina

(2008) K. L. Reddy et al.   NATURE

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

(2008) Paola Scaffidi et al.   NATURE CELL BIOLOGY

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

(2008) Ignacio Varela et al.   NATURE MEDICINE

Phenotype and Course of Hutchinson–Gilford Progeria Syndrome

(2008) Melissa A. Merideth et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

(2008) B. C. Capell et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2007) S YANG   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Involvement of xeroderma pigmentosum group A (XPA) in progeria arising from defective maturation of prelamin A

(2007) Yiyong Liu et al.   FASEB JOURNAL