Results 51 to 60 of about 3,816 (187)

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

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
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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

Nuclear Pore Complexes Cluster in Dysmorphic Nuclei of Normal and Progeria Cells during Replicative Senescence

Cells, 2021
Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disease caused by a mutation in LMNA. A G608G mutation in exon 11 of LMNA is responsible for most HGPS cases, generating a truncated protein called “progerin”.
Jennifer M. Röhrl, Rouven Arnold, Karima Djabali   +2 more
doaj   +1 more source

Nuclear import pathway key to rescuing dominant progerin phenotypes [PDF]

Science Signaling, 2018
Restoring a nuclear import pathway rescues pathological phenotypes of cells from HGPS patients (Larrieuet al., in 3 July 2018 issue).
openaire   +2 more sources

Development of an accelerated cellular model for early changes in Alzheimer’s disease

Scientific Reports, 2023
Alzheimer’s Disease (AD) is a leading cause of dementia characterized by amyloid plaques and neurofibrillary tangles, and its pathogenesis remains unclear.
Huijing Xue, Sylvester Gate, Emma Gentry, Wolfgang Losert, Kan Cao   +4 more
doaj   +1 more source

Mouse models for understanding the molecular mechanism of bone disease in Hutchinson-Gilford progeria syndrome [PDF]

, 2011
Aging is a complex process affecting all people. Intense research is applied to elucidate the biological basis of aging and disease that develop with aging.
Schmidt, Eva
core   +1 more source

Impact of MnTBAP and Baricitinib Treatment on Hutchinson–Gilford Progeria Fibroblasts

Pharmaceuticals, 2022
Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging disease. It is caused by a mutation in the LMNA gene, which results in a 50-amino-acid truncation of prelamin A.
Elena Vehns, Rouven Arnold, Karima Djabali   +2 more
doaj   +1 more source

Hutchinson Gilford Progeria Syndrome: A Therapeutic Approach via Adenoviral Delivery of CRISPR/cas Genome Editing System [PDF]

, 2014
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare human genetic disease caused by mutations in the LMNA gene. LMNA codes for structural components of the nuclear lamina. Alterations of nuclear lamina lead to a very variable class of diseases known as
ARANCIO, Walter, Genovese, SI, GIORDANO, Carla, PIZZOLANTI, Giuseppe   +3 more
core   +1 more source

A new connection between VHL and cancer threads through progerin [PDF]

Cell Cycle, 2013
A-type nuclear lamins, all encoded by the LMNA gene through alternative splicing, are targets for mutation in a wide range of rare diseases, including a dominant mutation that enhances production of lamin A splice variant progerin, causing Hutchinson-Gilford progeria syndrome (HGPS).1 Often ignored, however, is the complex and poorly understood ...
openaire   +2 more sources