|1.||Young, Stephen G: 15 articles (05/2015 - 10/2002)|
|2.||Fong, Loren G: 14 articles (05/2015 - 12/2004)|
|3.||Lattanzi, Giovanna: 9 articles (10/2015 - 06/2005)|
|4.||Yang, Shao H: 9 articles (05/2015 - 12/2004)|
|5.||Squarzoni, Stefano: 8 articles (10/2015 - 06/2005)|
|6.||Lattanzi, G: 8 articles (08/2011 - 11/2005)|
|7.||Capanni, Cristina: 7 articles (10/2015 - 06/2005)|
|8.||López-Otín, Carlos: 6 articles (10/2013 - 07/2005)|
|9.||Maraldi, N M: 6 articles (08/2011 - 11/2005)|
|10.||Columbaro, Marta: 5 articles (10/2015 - 06/2005)|
|1.||Progeria (Hutchinson Gilford Syndrome)
01/01/2009 - "Different prelamin A forms accumulate in human fibroblasts: a study in experimental models and progeria."
06/01/2013 - "In contrast to reported RD patients with LMNA mutations, LMNA p.R435C is not located at the cleavage site necessary for processing of prelamin A by ZMPSTE24 and leads to a distinct phenotype combining clinical features of Restrictive Dermopathy, Mandibuloacral Dysplasia and Hutchinson-Gilford Progeria. "
01/01/2013 - "Accumulation of prelamin A compromises NF-κB-regulated B-lymphopoiesis in a progeria mouse model."
01/01/2013 - "Mice deficient in Zmpste24, a metalloproteinase mutated in human progeria and involved in nuclear prelamin A maturation, recapitulate multiple features of ageing. "
01/01/2011 - "Accumulation of a truncated farnesylated prelamin A form, called progerin, is a hallmark of the severe premature ageing syndrome, Hutchinson-Gilford progeria. "
07/01/2015 - "The aim of this study is to use an experimental LMNA-lipodystrophy model based on human mesenchymal stem cell (hMSC)-derived adipocytes that accumulate prelamin A to gain deeper insights into the mechanisms governing these diseases. "
09/19/2008 - "These studies strongly support our hypothesis that HIV-PIs block prelamin A processing by directly affecting the enzymatic activity of ZMPSTE24, and in this way they may contribute to lipodystrophy in individuals undergoing HIV-PI treatment."
04/01/2012 - "The pathological mechanism by which prelamin A accumulation induces the lipodystrophy associated phenotypes remains unclear. "
11/01/2011 - "Similarly to humans, mice deficient in Zmpste24 accumulate prelamin A and display phenotypic features of accelerated aging, including lipodystrophy. "
07/01/2008 - "We also show that a combination of statins and aminobisphosphonates efficiently inhibits both farnesylation and geranylgeranylation of progerin and prelamin A and markedly improves the aging-like phenotypes of mice deficient in the metalloproteinase Zmpste24, including growth retardation, loss of weight, lipodystrophy, hair loss and bone defects. "
|3.||Familial Partial Lipodystrophy (Dunnigan Syndrome)
04/01/2015 - "Our results suggest that there is a relationship between mutated prelamin-A accumulation and the severity of the phenotypes in homozygous familial partial lipodystrophy type 2 patients who harbor the LMNA T655fsX49 mutation. "
01/01/2009 - "Site-dependent differences in both prelamin A and adipogenic genes in subcutaneous adipose tissue of patients with type 2 familial partial lipodystrophy."
10/01/2010 - "Prelamin A accumulation establishes a link with other types of familial lipodystrophies, as familial partial lipodystrophy."
10/01/2012 - "In this study, we show that the accumulation of wild-type prelamin A detected in restrictive dermopathy (RD), as well as the accumulation of mutated forms of prelamin A identified in familial partial lipodystrophy (FPLD) and mandibuloacral dysplasia (MADA), affect the nuclear localization of barrier-to-autointegration factor (BAF), a protein able to link lamin A precursor to chromatin remodeling functions. "
09/01/2009 - "Impairment of the lamin A maturation pathway causing lamin A precursor accumulation is linked to the development of rare diseases such as familial partial lipodystrophy, MADA (mandibuloacral dysplasia), the Werner syndrome, Hutchinson-Gilford progeria syndrome and RD (restrictive dermopathy). "
|4.||DNA Repair-Deficiency Disorders (Chromosome Instability Syndromes)
06/01/2013 - "The follow-up of the complete clinical course in the patient combined with functional studies showed for the first time that a progressive loss of lamin A rather than abnormal accumulation of prelamin A species could be a pathophysiological mechanism in progeroid laminopathies, which leads to DNA repair deficiency accompanied by advancing tissue degeneration. "
07/01/2015 - "Prelamin A accumulation causes mitochondrial dysfunction, endoplasmic reticulum stress, and altered lipid metabolism resembling a premature aging phenotype."
04/01/2014 - "We demonstrate that prelamin A-accumulating hMSCs have a premature aging phenotype which affects their functional competence in vivo. "
04/01/2014 - "Accumulation of progerin and prelamin A are the hallmark of a group of premature aging diseases but have also been found during normal cellular aging strongly suggesting similar mechanisms between healthy aging and LMNA-linked progeroid syndromes. "
01/01/2014 - "Taken together, these findings establish a functional link between prelamin A, HP1α, chromatin remodeling, DNA repair, and early senescence in Zmpste24-deficient mice, suggesting a potential therapeutic strategy for laminopathy-based premature aging via the intervention of HP1α. "
10/11/2013 - "Taken together, our data demonstrated the physical interaction between FAM96B and prelamin A, which may provide some clues to the mechanisms of prelamin A in premature aging. "
|1.||Lamin Type A (Lamin A)
|2.||Adenosine Triphosphate (ATP)
|4.||Staphylococcal Protein A (A, Protein)
|5.||Hutchinson Gilford progeria syndrome
|8.||Proteins (Proteins, Gene)
|10.||Protein Isoforms (Isoforms)