Article
Cardiac & Cardiovascular Systems
Leslie B. Gordon, Wendy Norris, Sarah Hamren, Robert Goodson, Jessica LeClair, Joseph Massaro, Asya Lyass, Ralph B. D'Agostino, Kelsey Tuminelli, Mark W. Kieran, Monica E. Kleinman
Summary: This study developed a plasma progerin assay to evaluate the quantity of progerin in HGPS patients and its response to treatment. The results showed that the plasma progerin levels were significantly elevated in HGPS patients and decreased after treatment, which was associated with patient survival.
Article
Biochemistry & Molecular Biology
Jon Macicior, Daniel Fernandez, Silvia Ortega-Gutierrez
Summary: Hutchinson-Gilford progeria syndrome (HGPS), also known as progeria, is a rare genetic disease that causes premature aging and significantly reduces life expectancy. Currently, there is only one approved drug for treating progeria, but its efficacy is limited. Progerin levels are believed to be the most important biomarker related to disease severity.
BIOORGANIC CHEMISTRY
(2024)
Article
Food Science & Technology
Jinsook Ahn, Tae-Gyun Woo, So-mi Kang, Inseong Jo, Jae-Sung Woo, Bum-Joon Park, Nam-Chul Ha
Summary: Nuclear lamin A/C is crucial for maintaining nuclear envelope structure, with Hutchinson-Gilford progeria syndrome (HGPS) being caused by gene mutation. Natural compounds like morin have been found to disrupt progerin-mediated nuclear deformation, offering potential benefits for HGPS patients and aging individuals.
JOURNAL OF FUNCTIONAL FOODS
(2021)
Review
Cell Biology
Huijing Xue, Abhirami Thaivalappil, Kan Cao
Summary: Methylene blue, the first fully man-made medicine, has a wide range of clinical applications and its anti-oxidative properties have brought new attention to this century-old drug. It can bypass Complex I/III activity in mitochondria and diminish oxidative stress to some degree, making it applicable for treating age-related conditions.
Article
Cell Biology
Karim Harhouri, Pierre Cau, Frank Casey, Koffi Mawuse Guedenon, Yassamine Doubaj, Lionel Van Maldergem, Gerardo Mejia-Baltodano, Catherine Bartoli, Annachiara De Sandre-Giovannoli, Nicolas Levy
Summary: Progeroid syndromes, such as Hutchinson-Gilford Progeria Syndrome (HGPS), are characterized by premature and accelerated aging. Most HGPS patients carry a genetic mutation in the LMNA gene, leading to the production of a truncated prelamin A called progerin. This study found that the proteasome inhibitor MG132 can induce the clearance of progerin and improve cellular phenotypes in HGPS-like patients' cells. These findings provide preclinical evidence for potential therapy for both HGPS-like and classical HGPS.
Review
Cell Biology
Olga Gomez, Giuliana Perini-Villanueva, Andrea Yuste, Jose Antonio Rodriguez-Navarro, Enric Poch, Eloy Bejarano
Summary: Autophagy is a crucial process in maintaining brain health by clearing dysfunctional cellular components, but its decline with age can lead to neurodegenerative disorders. Glycative stress, characterized by the accumulation of AGEs, negatively impacts brain health and may interfere with autophagic function. While autophagy can help remove harmful AGEs, excessive glycative stress may hinder its cytoprotective role in neurons and glial cells.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Amanda Sanchez-Lopez, Carla Espinos-Estevez, Cristina Gonzalez-Gomez, Pilar Gonzalo, Maria J. Andres-Manzano, Victor Fanjul, Raquel Riquelme-Borja, Magda R. Hamczyk, Alvaro Macias, Lara Del Campo, Emilio Camafeita, Jesus Vazquez, Anna Barkaway, Loic Rolas, Sussan Nourshargh, Beatriz Dorado, Ignacio Benedicto, Vicente Andres
Summary: The study showed that while targeting progerin in mice with mild symptoms produced more significant benefits, it is never too late to treat Hutchinson-Gilford progeria syndrome (HGPS). Restricting the suppression of progerin and restoration of lamin A to vascular smooth muscle cells and cardiomyocytes can effectively prevent vascular disease and normalize lifespan.
Review
Biochemistry & Molecular Biology
Margarita-Elena Papandreou, Nektarios Tavernarakis
Summary: Progressive accumulation of damaged cellular constituents contributes to age-related diseases. Autophagy is the main catabolic process, which recycles cellular material in a multitude of tissues and organs. Autophagy is activated upon various stimuli and involves precise interactions between receptors or adaptors and substrates to maintain specificity and accuracy. Polymorphisms in genes regulating selective autophagy have been linked to aging and age-associated disorders, suggesting potential therapeutic interventions to alleviate cellular autophagic cargo burden and associated pathologies.
Review
Medicine, Research & Experimental
Bulmaro Cisneros, Ian Garcia-Aguirre, Marlon De Ita, Isabel Arrieta-Cruz, Haydee Rosas-Vargas
Summary: Aging is the gradual decline of physical and psychological functions in humans, accompanied by the onset of chronic-degenerative diseases. The study of Hutchinson-Gilford progeria syndrome (HGPS) has provided insights into understanding the aging process. HGPS is caused by a genetic mutation that leads to the synthesis of progerin, a mutant version of lamin A. However, the mechanisms by which progerin induces detrimental alterations at the cellular and systemic levels are not fully understood.
ARCHIVES OF MEDICAL RESEARCH
(2023)
Review
Cell Biology
Bae-Hoon Kim, Yeon-Ho Chung, Tae-Gyun Woo, So-Mi Kang, Soyoung Park, Bum-Joon Park
Summary: HGPS is an extremely rare genetic disorder caused by the mutant protein progerin, which is expressed by the abnormal splicing of the LMNA gene. Studying HGPS could help uncover the causes of human aging and potentially improve patients' quality of life and extend their survival.
Article
Cell Biology
Ananth R. Srinivasan, Tracy T. Tran, Nancy M. Bonini
Summary: This study investigates the role of miR-34 in age-related phenotypes in the brain, finding that miR-34 regulates translation, protein aggregation, and autophagy. Additionally, the study suggests that miR-34 may target Lst8 and other genes involved in maintaining proteostasis and brain health.
Article
Cell Biology
Chien-Yuan Chen, Yung-Mei Chao, Ching-Chang Cho, Cheng-Sheng Chen, Wei-Yong Lin, Yi-Hung Chen, Marlene Cassar, Cecilia S. Lu, Jenq-Lin Yang, Julie Y. H. Chan, Suh-Hang H. Juo
Summary: This study demonstrated the role of miR-195 in aging and cognitive functions, and identified Sema3D as a direct target of miR-195 which is associated with age-associated neurodegeneration. The findings suggest that Sema3D could be a potential drug target for dementia treatment.
CELL COMMUNICATION AND SIGNALING
(2023)
Article
Biochemistry & Molecular Biology
Emilio Camafeita, Inmaculada Jorge, Jose Rivera-Torres, Vicente Andres, Jesus Vazquez
Summary: This study presents a reliable non-immunological method for quantifying the levels of wild-type lamin A and farnesylated progerin in cells from Hutchinson-Gilford progeria syndrome (HGPS) patients. This method, based on a targeted mass spectrometry approach and isotope-labeled internal standards, could be used to evaluate the efficacy of drugs inhibiting progerin farnesylation in ongoing clinical trials.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Medicine, Research & Experimental
Alvaro Gonzalez-Dominguez, Raul Montanez, Beatriz Castejon-Vega, Jessica Nunez-Vasco, Debora Lendines-Cordero, Chun Wang, Gabriel Mbalaviele, Jose M. Navarro-Pando, Elisabet Alcocer-Gomez, Mario D. Cordero
Summary: This study showed increased expression of NLRP3 inflammasome components in HGPS skin fibroblasts and lymphoblasts, as well as in hearts and livers of Zmpste24(-/-) mice. Inhibiting the NLRP3 inflammasome with MCC950 improved cellular phenotype, extended lifespan of progeroid animals, and reduced inflammation, suggesting a potential therapeutic approach for HGPS.
EMBO MOLECULAR MEDICINE
(2021)
Article
Geriatrics & Gerontology
Ramasamy Selvarani, Sabira Mohammed, Arlan Richardson
Summary: Rapamycin has shown potential to extend lifespan and treat age-related diseases in mice. Research should continue to focus on the effects of rapamycin in aging physiology and diseases, and bringing it into clinical applications.