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Published on: September 15, 2018
The atherogenic role of immune cells in familial hypercholesterolemia
Eskandar Taghizadeh1,2, Forough Taheri3, Seyed Mohammad Gheibi Hayat4
1Cellular and Molecular Research Center, Yasuj University of Medical Sciences, Yasuj, Iran.
Insights
Familial hypercholesterolemia (FH) involves immune cell dysfunction, accelerating atherosclerosis. Targeting these immune alterations offers new therapeutic strategies for FH patients.
Area of Science:
- Immunology
- Cardiovascular Medicine
- Genetics
Background:
- Familial hypercholesterolemia (FH) is an autosomal dominant genetic disorder impacting lipoprotein metabolism.
- It is characterized by elevated low-density lipoprotein cholesterol (LDL-C), promoting early atherosclerosis and heart disease.
- Immune cells, including monocytes, macrophages, neutrophils, T cells, and B cells, are crucial in atherogenesis.
Purpose of the Study:
- To review the specific roles of immune system alterations in FH.
- To explore how immune cells contribute to FH pathogenesis.
- To identify immune cells as potential therapeutic targets in FH.
Main Methods:
- Review of existing literature on FH and immune responses.
- Analysis of immune cell behavior in the context of high cholesterol accumulation.
- Examination of pro-atherosclerotic activities of immune cells in FH.
Main Results:
- Immune cells exhibit unique pro-atherosclerotic activities in FH, from leukocyte recruitment to plaque rupture.
- High cholesterol crystals and oxidized LDL (oxLDL) in FH vessels trigger abnormal immune cell mobilization.
- Chronic cholesterol exposure exacerbates immune cell dysfunction and inflammation in FH.
Conclusions:
- Immune system alterations are central to FH pathophysiology.
- Specific immune cell dysfunctions in FH contribute to accelerated atherosclerosis.
- Targeting immune cells presents a promising avenue for novel FH therapies.
Abstract:
Familial hypercholesterolemia (FH) is an autosomal dominant disorder of lipoprotein metabolism that mainly occurs due to mutations in the low-density lipoprotein receptor gene and is characterized by increased levels of low-density lipoprotein cholesterol, leading to accelerated atherogenesis and premature coronary heart disease. Both innate and adaptive immune responses, which mainly include monocytes, macrophages, neutrophils, T lymphocytes, and B lymphocytes, have been shown to play a key role for the initiation and progression of atherogenesis in the general population. In FH patients, these immune cells have been suggested to play specific pro-atherosclerotic activities, from the initial leukocyte recruitment to plaque rupture. In fact, the accumulation of cholesterol crystals and oxLDL in the vessels in FH patients is particularly high, with consequent abnormal mobilization of immune cells and secretion of various pro-inflammatory and chemokines. In addition, cholesterol accumulation in immune cells is exaggerated with chronic exposure to relevant pro-atherosclerotic triggers. The topics considered in this review may provide a more specific focus on the immune system alterations in FH and open new insights toward immune cells as potential therapeutic targets in FH.
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