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A Familial Hypercholesterolemia Human Liver Chimeric Mouse Model Using Induced Pluripotent Stem Cell-derived Hepatocytes
Published on: September 15, 2018
Circulating progenitor cells in patients with familial hypercholesterolemia
P B Sandesara1, V Ramjee2, N Ghasemzadeh1
1Emory University School of Medicine, 1365 Clifton Road NE, Atlanta, Georgia 30322.
Familial hypercholesterolemia patients show higher baseline circulating progenitor cells (CPCs), indicating activated repair mechanisms. However, a single apheresis session did not alter CPC levels in these individuals.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Hematology
Background:
- Familial hypercholesterolemia (FH) is a genetic disorder characterized by extremely high low-density lipoprotein cholesterol (LDL-C) levels, accelerating atherosclerosis.
- Lipoprotein apheresis is a key treatment for FH, reducing cardiovascular events.
- Circulating progenitor cells (CPCs) reflect vascular health and reparative capacity; lower levels correlate with poorer outcomes.
Purpose of the Study:
- To evaluate the short-term impact of lipoprotein apheresis on CPC levels in FH patients undergoing stable therapy.
- To test the hypothesis that apheresis enhances vascular repair by mobilizing CPCs, in addition to lipid reduction.
Main Methods:
- Eight FH patients (1 homozygous, 7 heterozygous) on stable apheresis therapy for ≥3 months had CPCs measured pre- and post-apheresis.
- Results were compared to age-matched hyperlipidemic (HLP) patients on statins and healthy controls.
Main Results:
- FH patients exhibited significantly higher baseline levels of CD34+/CD133+ and CD34+/CD133+/CXCR4+ CPCs than HLP and healthy subjects.
- No significant change in CPC counts was observed two hours after a single lipoprotein apheresis session.
Conclusions:
- Elevated baseline CPC counts in FH patients suggest an inherent activation of vascular repair mechanisms in this high-risk group.
- Further research is required to understand the dynamics of CPC levels in FH versus HLP populations over time.
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