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References from Abnormal nuclear morphology in vivo in mouse hair follicle fibroblasts expressing progerin. Local targets link to admitted publications; unresolved targets remain external evidence.
The Hutchinson-Gilford progeria syndrome. Report of 4 cases and review of the literature
10.1016/s0022-3476(72)80229-4 · 1972 · External reference
Phenotype and course of Hutchinson-Gilford progeria syndrome
10.1056/nejmoa0706898 · 2008 · External reference
Lamin a truncation in Hutchinson-Gilford progeria
10.1126/science.1084125 · 2003 · External reference
Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome
10.1038/nature01629 · 2003 · External reference
cDNA sequencing of nuclear lamins A and C reveals primary and secondary structural homology to intermediate filament proteins
10.1073/pnas.83.17.6450 · 1986 · External reference
Homologies in both primary and secondary structure between nuclear envelope and intermediate filament proteins
10.1038/319463a0 · 1986 · External reference
Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C
10.1016/s0021-9258(19)85424-8 · 1993 · External reference
The molecular architecture of lamins in somatic cells
10.1038/nature21382 · 2017 · External reference
Isoprenylation is required for the processing of the lamin A precursor
10.1083/jcb.110.5.1489 · 1990 · External reference
The processing pathway of prelamin A
10.1242/jcs.107.1.61 · 1994 · External reference
Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect
10.1073/pnas.192460799 · 2002 · External reference
Defective prelamin A processing and muscular and adipocyte alterations in Zmpste24 metalloproteinase-deficient mice
10.1038/ng871 · 2002 · External reference
Prelamin A endoproteolytic processing in vitro by recombinant Zmpste24
10.1042/bj20041359 · 2005 · External reference
Aging of Hutchinson-Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis
10.1016/j.exger.2004.02.002 · 2004 · External reference
Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome
10.1073/pnas.0402943101 · 2004 · External reference
Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress
10.1186/1471-2121-6-27 · 2005 · External reference
Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation
10.1073/pnas.0504641102 · 2005 · External reference
Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes
10.1073/pnas.0505767102 · 2005 · External reference
Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome
10.1073/pnas.0506001102 · 2005 · External reference
Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome
10.1073/pnas.0503712102 · 2005 · External reference
Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition
10.1093/hmg/ddi326 · 2005 · External reference
A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation
10.1172/jci28968 · 2006 · External reference
A farnesyltransferase inhibitor prevents both the onset and late progression of cardiovascular disease in a progeria mouse model
10.1073/pnas.0807840105 · 2008 · External reference
Clinical trial of a farnesyltransferase inhibitor in children with Hutchinson-Gilford progeria syndrome
10.1073/pnas.1202529109 · 2012 · External reference
Association of lonafarnib treatment vs no treatment with mortality rate in patients with Hutchinson-Gilford progeria syndrome
10.1001/jama.2018.3264 · 2018 · External reference
FDA approval summary for lonafarnib (Zokinvy) for the treatment of Hutchinson-Gilford progeria syndrome and processing-deficient progeroid laminopathies
10.1016/j.gim.2022.11.003 · 2023 · External reference
Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome
10.1038/nm1204 · 2005 · External reference
Splicing-directed therapy in a new mouse model of human accelerated aging
10.1126/scitranslmed.3002847 · 2011 · External reference
Modulation of LMNA splicing as a strategy to treat prelamin A diseases
10.1172/jci85908 · 2016 · External reference
Transcriptional profiling of Hutchinson-Gilford progeria patients identifies primary target pathways of progerin
10.1080/19491034.2025.2611484 · 2026 · External reference
Lamin A/C assembly defects in Emery-Dreifuss muscular dystrophy can be regulated by culture medium composition
10.1016/j.nmd.2006.03.014 · 2006 · External reference
Materials and extracellular matrix rigidity highlighted in tissue damages and diseases: implication for biomaterials design and therapeutic targets
2022 · External reference
Remodeling and homeostasis of the extracellular matrix: implications for fibrotic diseases and cancer
10.1242/dmm.004077 · 2011 · External reference
Nuclear lamin-A scales with tissue stiffness and enhances matrix-directed differentiation
10.1126/science.1240104 · 2013 · External reference
Nuclear membrane ruptures underlie the vascular pathology in a mouse model of Hutchinson-Gilford progeria syndrome
10.1172/jci.insight.151515 · 2021 · External reference
Disrupting the LINC complex in smooth muscle cells reduces aortic disease in a mouse model of Hutchinson-Gilford progeria syndrome
10.1126/scitranslmed.aat7163 · 2018 · External reference
Progerin in muscle leads to thermogenic and metabolic defects via impaired calcium homeostasis
10.1111/acel.13090 · 2020 · External reference
Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin
10.1093/hmg/ddn136 · 2008 · External reference
Blocking protein farnesylation improves nuclear shape abnormalities in keratinocytes of mice expressing the prelamin A variant in Hutchinson-Gilford progeria syndrome
10.4161/nucl.1.5.12972 · 2010 · External reference
Short promoters in viral vectors drive selective expression in mammalian inhibitory neurons, but do not restrict activity to specific inhibitory cell-types
2009 · External reference
Distinct fibroblast lineages determine dermal architecture in skin development and repair
10.1038/nature12783 · 2013 · External reference
Characterization of the platelet-derived growth factor receptor-α-positive cell lineage during murine late lung development
10.1152/ajplung.00272.2014 · 2015 · External reference
Reflections on how wound healing-promoting effects of the hair follicle can be translated into clinical practice
10.1111/exd.12521 · 2015 · External reference
Harnessing the secretome of hair follicle fibroblasts to accelerate ex vivo healing of human skin wounds
10.1016/j.jid.2019.09.019 · 2020 · External reference
Imbalanced nucleocytoskeletal connections create common polarity defects in progeria and physiological aging
10.1073/pnas.1809683116 · 2019 · External reference
Hair follicle dermal papilla cells at a glance
10.1242/jcs.082446 · 2011 · External reference
A comprehensive guide for the recognition and classification of distinct stages of hair follicle morphogenesis
10.1046/j.1523-1747.1999.00740.x · 1999 · External reference
A comprehensive guide for the accurate classification of murine hair follicles in distinct hair cycle stages
10.1046/j.0022-202x.2001.01377.x · 2001 · External reference
Specific expression in mouse mesoderm- and neural crest-derived tissues of a human PDGFRA promoter/lacZ transgene
10.1016/s0925-4773(97)00190-1 · 1998 · External reference
Progressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome
10.1073/pnas.0600012103 · 2006 · External reference
Targeted transgenic expression of the mutation causing Hutchinson-Gilford progeria syndrome leads to proliferative and degenerative epidermal disease
10.1242/jcs.022913 · 2008 · External reference
Embryonic expression of the common progeroid lamin A splice mutation arrests postnatal skin development
10.1111/acel.12173 · 2014 · External reference
Long term breeding of the Lmna G609G progeric mouse: characterization of homozygous and heterozygous models
10.1016/j.exger.2019.110784 · 2020 · External reference
Transient expression of an adenine base editor corrects the Hutchinson-Gilford progeria syndrome mutation and improves the skin phenotype in mice
10.1038/s41467-022-30800-y · 2022 · External reference
Inflammation and fibrosis in progeria: organ-specific responses in an HGPS mouse model
10.3390/ijms25179323 · 2024 · External reference
Stem cell depletion in Hutchinson-Gilford progeria syndrome
10.1111/j.1474-9726.2011.00743.x · 2011 · External reference
Fibroblast clones from patients with Hutchinson-Gilford progeria can senesce despite the presence of telomerase
10.1016/j.exger.2003.12.015 · 2004 · External reference
Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-lamin A G608G antibody
10.1073/pnas.0511133103 · 2006 · External reference
Suppression of proliferative defects associated with processing-defective lamin A mutants by hTERT or inactivation of p53
10.1091/mbc.e08-05-0492 · 2008 · External reference
Cardiac and skeletal muscle manifestations in the G608G mouse model of Hutchinson-Gilford progeria syndrome
10.1111/acel.14259 · 2024 · External reference