Mouse models of laminopathies (Q26829927)

Julia E. Kieckhaefer

series ordinal

2

1 reference

Kan Cao

series ordinal

3

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language of work or name

1 reference

February 2013

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1 reference

12

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PubMed

page(s)

2-10

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PubMed

issue

1

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

PubMed

cites work

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Differential temporal and spatial progerin expression during closure of the ductus arteriosus in neonates

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

A lamin A protein isoform overexpressed in Hutchinson-Gilford progeria syndrome interferes with mitosis in progeria and normal cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Human laminopathies: nuclei gone genetically awry

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Direct synthesis of lamin A, bypassing prelamin a processing, causes misshapen nuclei in fibroblasts but no detectable pathology in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Conditional gene expression in the epidermis of transgenic mice using the tetracycline-regulated transactivators tTA and rTA linked to the keratin 5 promoter

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Automated image analysis of nuclear shape: what can we learn from a prematurely aged cell?

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

The nuclear lamina comes of age.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Functional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

B-type lamins and their elusive roles in metazoan cell proliferation and senescence

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Mouse B-type lamins are required for proper organogenesis but not by embryonic stem cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Post-natal myogenic and adipogenic developmental: defects and metabolic impairment upon loss of A-type lamins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Decreased bone formation and osteopenia in lamin a/c-deficient mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

The laminopathies: nuclear structure meets disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Expression of an LMNA-N195K variant of A-type lamins results in cardiac conduction defects and death in mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Reversible phenotype in a mouse model of Hutchinson-Gilford progeria syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Lamin A-dependent nuclear defects in human aging

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Mouse models of the laminopathies.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Nuclear lamins: their structure, assembly, and interactions

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

A mitotic lamin B matrix induced by RanGTP required for spindle assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Lamin B1 is required for mouse development and nuclear integrity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Lamin A/C deficiency is associated with fat infiltration of muscle and bone

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

Defective prelamin A processing and muscular and adipocyte alterations in Zmpste24 metalloproteinase-deficient mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

7 January 2021

Mouse model carrying H222P- Lmna mutation develops muscular dystrophy and dilated cardiomyopathy similar to human striated muscle laminopathies

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

7 January 2021

Pathology and nuclear abnormalities in hearts of transgenic mice expressing M371K lamin A encoded by an LMNA mutation causing Emery-Dreifuss muscular dystrophy

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

7 January 2021

Stem cell depletion in Hutchinson-Gilford progeria syndrome

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

7 January 2021

The lethal phenotype of a homozygous nonsense mutation in the lamin A/C gene

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

7 January 2021

Targeted transgenic expression of the mutation causing Hutchinson-Gilford progeria syndrome leads to proliferative and degenerative epidermal disease

1 reference

https://api.crossref.org/works/10.1111%2FACEL.12021

7 January 2021