Hutchinson-Gilford progeria syndrome: Cardiovascular manifestations and treatment.
Mech Ageing Dev
; 216: 111879, 2023 Dec.
Article
en En
| MEDLINE
| ID: mdl-37832833
ABSTRACT
Hutchinson-Gilford progeria syndrome (HGPS), also known as hereditary progeria syndrome, is caused by mutations in the LMNA gene and the expression of progerin, which causes accelerated aging and premature death, with most patients dying of heart failure or other cardiovascular complications in their teens. HGPS patients are able to exhibit cardiovascular phenotypes similar to physiological aging, such as extensive atherosclerosis, smooth muscle cell loss, vascular lesions, and electrical and functional abnormalities of the heart. It also excludes the traditional risk causative factors of cardiovascular disease, making HGPS a new model for studying aging-related cardiovascular disease. Here, we analyzed the pathogenesis and pathophysiological characteristics of HGPS and the relationship between HGPS and cardiovascular disease, provided insight into the molecular mechanisms of cardiovascular disease pathogenesis in HGPS patients and treatment strategies for this disease. Moreover, we summarize the disease models used in HGPS studies to improve our understanding of the pathological mechanisms of cardiovascular aging in HGPS patients.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Contexto en salud:
2_ODS3
Problema de salud:
2_muertes_prematuras_enfermedades_notrasmisibles
Asunto principal:
Progeria
/
Enfermedades Cardiovasculares
/
Sistema Cardiovascular
/
Aterosclerosis
Límite:
Adolescent
/
Humans
Idioma:
En
Revista:
Mech Ageing Dev
Año:
2023
Tipo del documento:
Article
País de afiliación:
China