A 42-year-old Indian man presents with severe hypercholesterolaemia (LDL 520 mg/dL), premature coronary artery disease at age 38, and Achilles tendon xanthomas. Genetic testing reveals a heterozygous PCSK9 gain-of-function mutation. Which feature best distinguishes this PCSK9-FH phenotype from LDLR-FH?

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