Results 41 to 50 of about 3,871 (195)

The telomeric protein AKTIP interacts with A- and B-type lamins and is involved in regulation of cellular senescence [PDF]

, 2016
AKTIP is a shelterin-interacting protein required for replication of telomeric DNA. Here, we show that AKTIP biochemically interacts with A- and B-type lamins and affects lamin A, but not lamin C or B, expression.
Astrologo, Letizia, Biroccio, Annamaria, Burla, Romina, Carcuro, Mariateresa, Crescenzi, Marco, Cundari, Enrico, D'Apice, Maria Rosaria, Fratini, Federica, Gatti, Maurizio, LA TORRE, Mattia, Lattanzi, Giovanna, Raffa, GRAZIA DANIELA, Raimondo, Domenico, Saggio, Isabella, Spitalieri, Paola   +14 more
core   +2 more sources

Mammalian telomeres and their partnership with lamins [PDF]

, 2016
Chromosome ends are complex structures, which require a panel of factors for their elongation, replication, and protection. We describe here the mechanics of mammalian telomeres, dynamics and maintainance in relation to lamins.
BURLA, ROMINA, LA TORRE, MATTIA, SAGGIO, Isabella   +2 more
core   +1 more source

Identification of differential protein interactors of lamin A and progerin [PDF]

Nucleus, 2010
The nuclear lamina is an interconnected meshwork of intermediate filament proteins underlying the nuclear envelope. The lamina is an important regulator of nuclear structural integrity as well as nuclear processes, including transcription, DNA replication and chromatin remodeling. The major components of the lamina are A- and B-type lamins.
Kubben, N, Voncken, JW, Demmers, Jeroen, Calis, C, van Almen, G, Pinto, Y, Misteli, T   +6 more
openaire   +3 more sources

Methionine Restriction Extends Lifespan in Progeroid Mice and Alters Lipid and Bile Acid Metabolism [PDF]

, 2018
C.L.-O. is supported by grants from the European Union (ERC-2016-ADG, DeAge); Ministerio de Economía y Competitividad (MINECO/FEDER: SAF2014-52413-R and SAF2017-87655-R); Instituto de Salud Carlos III (RTICC); Progeria Research Foundation (PRF2016-66 ...
Bárcena Fernández, Clea, Durand, S., Fernández García, María Teresa, Garabaya Fernández, Cecilia, Kroemer, Guido, López Otín, Carlos, Mariño García, Guillermo, Mayoral García, Pablo, Menéndez Caravia, Xurde, Moral Quirós, Pedro, Pérez Freije, José María, Rodríguez, Francisco   +11 more
core   +3 more sources

Sulforaphane enhances progerin clearance in Hutchinson–Gilford progeria fibroblasts [PDF]

Aging Cell, 2014
SummaryHutchinson–Gilford progeria syndrome (HGPS, OMIM 176670) is a rare multisystem childhood premature aging disorder linked to mutations in the LMNA gene. The most common HGPS mutation is found at position G608G within exon 11 of the LMNA gene. This mutation results in the deletion of 50 amino acids at the carboxyl‐terminal tail of prelamin A, and ...
Gabriel, Diana, Roedl, Daniela, Djabali, Karima, Gordon, Leslie   +3 more
openaire   +4 more sources

Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome [PDF]

, 2019
Hutchinson–Gilford progeria syndrome (HGPS) is a genetic disorder characterized by premature aging features. Cells from HGPS patients express progerin, a truncated form of Lamin A, which perturbs cellular homeostasis leading to nuclear shape alterations,
Aguado J., Cancila V., d'Adda di Fagagna F., Dreesen O., Eriksson M., Jones-Weinert C. W., Lattanzi G., Ong P. F., Revechon G., Rossiello F., Sola-Carvajal A., Tripodo C., Wallen Arzt E.   +12 more
core   +1 more source

Progerin modulates the IGF-1R/Akt signaling involved in aging

Science Advances, 2022
Progerin, a product of LMNA mutation, leads to multiple nuclear abnormalities in patients with Hutchinson-Gilford progeria syndrome (HGPS), a devastating premature aging disorder. Progerin also accumulates during physiological aging.
Bo Jiang, Xuan Wu, Fang Meng, Limiao Si, Sunrun Cao, Yuqing Dong, Huayi Sun, Mengzhu Lv, Hongde Xu, Ning Bai, Qiqiang Guo, Xiaoyu Song, Yang Yu, Wendong Guo, Fei Yi, Tingting Zhou, Xiaoman Li, Yanling Feng, Zhuo Wang, Dan Zhang, Yi Guan, Mengtao Ma, Jingwei Liu, Xining Li, Weidong Zhao, Baohua Liu, Toren Finkel, Liu Cao   +27 more
openaire   +2 more sources

Genomic instability and DNA replication defects in progeroid syndromes [PDF]

, 2018
Progeroid syndromes induced by mutations in lamin A or in its interactors – named progeroid laminopathies – are model systems for the dissection of the molecular pathways causing physio- logical and premature aging.
Chiara Merigliano, Fiammetta Vernì, Isabella Saggio, Mattia La Torre, Romina Burla   +4 more
core   +1 more source

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

PLoS ONE, 2007
Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder characterized by accelerated aging and early death, frequently from stroke or coronary artery disease. 90% of HGPS cases carry the LMNA G608G (GGC>GGT) mutation within exon 11 of
Dayle McClintock, Desiree Ratner, Meepa Lokuge, David M Owens, Leslie B Gordon, Francis S Collins, Karima Djabali   +6 more
doaj   +1 more source

Increasing the length of progerin's isoprenyl anchor does not worsen bone disease or survival in mice with Hutchinson-Gilford progeria syndrome

Journal of Lipid Research, 2009
Hutchinson-Gilford progeria syndrome (HGPS) is caused by the synthesis of a truncated prelamin A, commonly called progerin, that contains a carboxyl-terminal farnesyl lipid anchor.
Brandon S.J. Davies, Shao H. Yang, Emily Farber, Roger Lee, Suzanne B. Buck, Douglas A. Andres, H. Peter Spielmann, Brian J. Agnew, Fuyuhiko Tamanoi, Loren G. Fong, Stephen G. Young   +10 more
doaj   +1 more source