The available medications used to treat HoFH are targeted at reducing circulating levels of total and LDL-cholesterol. These measures can retard the progression of cardiovascular disease, however, they are unlikely to regress existing disease due to years of cholesterol accumulation in the vessel walls and therefore cannot adequately reduce the existing risk for an ischemic event. HDL has multiple actions that could lead to plaque stabilization and regression, such as rapid removal of large quantities of cholesterol from the vasculature, improvement in endothelial function, protection against oxidative damage and reduction in inflammation. This study will assess the effects of CER-001, a recombinant human Apo-A-1 based HDL mimetic, on indices of atherosclerotic plaque progression and regression as assessed by 3Tesla MRI measurements in patients with HoFH.

Inclusion Criteria:

• Male or female subject 12 years or older
• Subject presents with Homozygous FH

Exclusion Criteria:

• Weight >100 kg
• Subjects with significant health problems in the recent past including blood disorders, cancer, or digestive problems
• Female subjects of child-bearing potential
• Known major hematologic, renal , hepatic, metabolic, gastrointestinal or endocrine dysfunction
• Contraindication to MRI scanning that would preclude the use of contrast-enhanced 3TMRI