Loss-of-function PCSK9 mutants evade the unfolded protein response sensor GRP78 and fail to induce endoplasmic reticulum stress when retained

Evolocumab and Clinical Outcomes in Patients with Cardiovascular Disease

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Endoplasmic Reticulum Stress and Ca2+Depletion Differentially Modulate the Sterol Regulatory Protein PCSK9 to Control Lipid Metabolism

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Srebp2: A master regulator of sterol and fatty acid synthesis

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The endoplasmic reticulum: structure, function and response to cellular signaling

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RNA therapeutics inactivate PCSK9 by inducing a unique intracellular retention form

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GRP94 Regulates Circulating Cholesterol Levels through Blockade of PCSK9-Induced LDLR Degradation

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PCSK9 gene mutations and low-density lipoprotein cholesterol

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ER-associated degradation: Protein quality control and beyond

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Orchestration of secretory protein folding by ER chaperones

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ER stress-induced cell death mechanisms

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Liver-specific knockout of GRP94 in mice disrupts cell adhesion, activates liver progenitor cells, and accelerates liver tumorigenesis

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Endoplasmic Reticulum Stress Sensing in the Unfolded Protein Response

(2013) B. M. Gardner et al.   Cold Spring Harbor Perspectives in Biology

Identification and characterization of new gain-of-function mutations in the PCSK9 gene responsible for autosomal dominant hypercholesterolemia

(2012) Marianne Abifadel et al.   ATHEROSCLEROSIS

Anti-PCSK9 therapies for the treatment of hypercholesterolemia

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Lipoproteins, cholesterol homeostasis and cardiac health

(2012) Tyler F. Daniels et al.   International Journal of Biological Sciences

Loss- and Gain-of-function PCSK9 Variants

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GRP94: An HSP90-like protein specialized for protein folding and quality control in the endoplasmic reticulum

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Novel Loss-of-Function PCSK9 Variant Is Associated with Low Plasma LDL Cholesterol in a French-Canadian Family and with Impaired Processing and Secretion in Cell Culture

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Coordination of Lipid Metabolism in Membrane Biogenesis

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The Unfolded Protein Response in Health and Disease

(2009) Richard C. Austin   ANTIOXIDANTS & REDOX SIGNALING

Mutations in NOTCH3 cause the formation and retention of aggregates in the endoplasmic reticulum, leading to impaired cell proliferation

(2009) Keikichi Takahashi et al.   HUMAN MOLECULAR GENETICS

Membrane expansion alleviates endoplasmic reticulum stress independently of the unfolded protein response

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Dominant pro-vasopressin mutants that cause diabetes insipidus form disulfide-linked fibrillar aggregates in the endoplasmic reticulum

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Proprotein convertase subtilisin/kexin type 9 (PCSK9): Hepatocyte-specific low-density lipoprotein receptor degradation and critical role in mouse liver regeneration

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Characterization of novel mutations in the catalytic domain of the PCSK9 gene

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Defective Protein Folding and Intracellular Retention of Thyroglobulin-R19K Mutant as a Cause of Human Congenital Goiter

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