Familial Hypercholesterolemia

Cardiovascular disease (CVD) in heterozygous familial hypercholesterolemia (HeFH), the most frequent monogenic disorder of human metabolism, is largely driven by low-density lipoprotein (LDL) cholesterol concentrations. Since the CVD rate differs considerably in this population, beyond the lifetime...

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Detalhes bibliográficos
Autores: Pedro Botet Montoya, Juan|||0000-0001-7366-6821, Climent, Elisenda|||0000-0001-6831-8224, Benaiges, David|||0000-0001-5411-364X
Tipo de documento: artigo
Data de publicação:2021
País:España
Recursos:Universitat Autònoma de Barcelona
Repositório:Dipòsit Digital de Documents de la UAB
Idioma:inglês
OAI Identifier:oai:ddd.uab.cat:293133
Acesso em linha:https://ddd.uab.cat/record/293133
https://dx.doi.org/urn:doi:10.3390/biomedicines9070810
Access Level:Acceso aberto
Palavra-chave:Cardiovascular disease
Epigenetics
Familial hypercholesterolemia
Genetics
HDL functionality
High density lipoproteins
Descrição
Resumo:Cardiovascular disease (CVD) in heterozygous familial hypercholesterolemia (HeFH), the most frequent monogenic disorder of human metabolism, is largely driven by low-density lipoprotein (LDL) cholesterol concentrations. Since the CVD rate differs considerably in this population, beyond the lifetime LDL cholesterol vascular accumulation, other classical risk factors are involved in the high cardiovascular risk of HeFH. Among other lipoprotein disturbances, alterations in the phenotype and functionality of high-density lipoproteins (HDL) have been described in HeFH patients, contributing to the presence and severity of CVD. In fact, HDL are the first defensive barrier against the burden of high LDL cholesterol levels owing to their contribution to reverse cholesterol transport as well as their antioxidant and anti-inflammatory properties, among others. In this context, the present narrative review aimed to focus on quantitative and qualitative abnormalities in HDL particles in HeFH, encompassing metabolic, genetic and epigenetic aspects.