Literature DB >> 27218270

PCSK9 R46L Loss-of-Function Mutation Reduces Lipoprotein(a), LDL Cholesterol, and Risk of Aortic Valve Stenosis.

Anne Langsted1, Børge G Nordestgaard1, Marianne Benn1, Anne Tybjærg-Hansen1, Pia R Kamstrup1.   

Abstract

CONTEXT: Novel, low-density lipoprotein (LDL) cholesterol-lowering proprotein convertase subtilisin/kexin type-9 (PCSK9) inhibitors also lower lipoprotein(a) levels, but the effect on aortic valve stenosis and myocardial infarction is unknown.
OBJECTIVE: We tested the hypothesis that the PCSK9 R46L loss-of-function mutation is associated with lower levels of lipoprotein(a) and with reduced risk of aortic valve stenosis and myocardial infarction.
DESIGN: We used two prospective cohort studies of the general population and one patient-based cohort.
SETTING: Cohort studies selected at random individuals of Danish descent. PARTICIPANTS: We studied 103 083 individuals from the Copenhagen General Population Study, the Copenhagen City Heart Study, and the Copenhagen Ischemic Heart Disease Study. MAIN OUTCOME MEASURES: Lipoprotein(a), LDL cholesterol, and PCSK9 R46L genotype and diagnoses of aortic valve stenosis and myocardial infarction from national registries; lipoprotein(a) was measured from 49,617 individuals.
RESULTS: Median (interquartile range) lipoprotein(a) levels were 10 (5-30) mg/dl for PCSK9 R46L noncarriers, 9 (4-32) mg/dl for heterozygotes, and 8 (4-42) mg/dl for homozygotes (trend P = .02). The corresponding values for LDL cholesterol levels were 124 (101-147) mg/dl, 104 (85-132) mg/dl, and 97 (85-128) mg/dl, respectively (trend P = 2 × 10(-52)). PCSK9 R46L carriers vs noncarriers had an age- and sex-adjusted odds ratio of 0.64 (95% confidence interval, 0.44-0.95) for aortic valve stenosis, 0.77 (0.65-0.92) for myocardial infarction, and 0.76 (0.64-0.89) for aortic valve stenosis or myocardial infarction.
CONCLUSIONS: PCSK9 R46L carriers have lower levels of lipoprotein(a) and LDL cholesterol as well as reduced risk of aortic valve stenosis and myocardial infarction. This indirectly suggests that PCSK9 inhibitors may have a role in patients with aortic valve stenosis.

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Year:  2016        PMID: 27218270     DOI: 10.1210/jc.2016-1206

Source DB:  PubMed          Journal:  J Clin Endocrinol Metab        ISSN: 0021-972X            Impact factor:   5.958


  31 in total

1.  Carriers of the PCSK9 R46L Variant Are Characterized by an Antiatherogenic Lipoprotein Profile Assessed by Nuclear Magnetic Resonance Spectroscopy-Brief Report.

Authors:  Rutger Verbeek; Marjorie Boyer; S Matthijs Boekholdt; G Kees Hovingh; John J P Kastelein; Nicholas Wareham; Kay-Tee Khaw; Benoit J Arsenault
Journal:  Arterioscler Thromb Vasc Biol       Date:  2016-11-17       Impact factor: 8.311

Review 2.  Can Lp(a) Lowering Against Background Statin Therapy Really Reduce Cardiovascular Risk?

Authors:  Željko Reiner
Journal:  Curr Atheroscler Rep       Date:  2019-03-07       Impact factor: 5.113

3.  Loss-of-function mutation in Mirta22/Emc10 rescues specific schizophrenia-related phenotypes in a mouse model of the 22q11.2 deletion.

Authors:  Anastasia Diamantopoulou; Ziyi Sun; Jun Mukai; Bin Xu; Karine Fenelon; Maria Karayiorgou; Joseph A Gogos
Journal:  Proc Natl Acad Sci U S A       Date:  2017-07-10       Impact factor: 11.205

Review 4.  NHLBI Working Group Recommendations to Reduce Lipoprotein(a)-Mediated Risk of Cardiovascular Disease and Aortic Stenosis.

Authors:  Sotirios Tsimikas; Sergio Fazio; Keith C Ferdinand; Henry N Ginsberg; Marlys L Koschinsky; Santica M Marcovina; Patrick M Moriarty; Daniel J Rader; Alan T Remaley; Gissette Reyes-Soffer; Raul D Santos; George Thanassoulis; Joseph L Witztum; Simhan Danthi; Michelle Olive; Lijuan Liu
Journal:  J Am Coll Cardiol       Date:  2018-01-16       Impact factor: 24.094

5.  PCSK9 loss-of-function variants and Lp(a) phenotypes among black US adults.

Authors:  Matthew T Mefford; Santica M Marcovina; Vera Bittner; Mary Cushman; Todd M Brown; Michael E Farkouh; Sotirios Tsimikas; Keri L Monda; J Antonio G López; Paul Muntner; Robert S Rosenson
Journal:  J Lipid Res       Date:  2019-09-11       Impact factor: 5.922

Review 6.  The current landscape of lipoprotein(a) in calcific aortic valvular disease.

Authors:  Grace Hsieh; Theresa Rizk; Adam N Berman; David W Biery; Ron Blankstein
Journal:  Curr Opin Cardiol       Date:  2021-09-01       Impact factor: 2.108

7.  Effects of the coronary artery disease associated LPA and 9p21 loci on risk of aortic valve stenosis.

Authors:  Teresa Trenkwalder; Christopher P Nelson; Muntaser D Musameh; Ify R Mordi; Thorsten Kessler; Costanza Pellegrini; Radoslaw Debiec; Tobias Rheude; Viktor Lazovic; Lingyao Zeng; Andreas Martinsson; J Gustav Smith; Jesper R Gådin; Anders Franco-Cereceda; Per Eriksson; Jonas B Nielsen; Sarah E Graham; Cristen J Willer; Kristian Hveem; Adnan Kastrati; Peter S Braund; Colin N A Palmer; Amparo Aracil; Oliver Husser; Wolfgang Koenig; Heribert Schunkert; Chim C Lang; Christian Hengstenberg; Nilesh J Samani
Journal:  Int J Cardiol       Date:  2018-11-17       Impact factor: 4.164

Review 8.  Potential Causality and Emerging Medical Therapies for Lipoprotein(a) and Its Associated Oxidized Phospholipids in Calcific Aortic Valve Stenosis.

Authors:  Sotirios Tsimikas
Journal:  Circ Res       Date:  2019-02       Impact factor: 17.367

Review 9.  Innate and adaptive immunity in cardiovascular calcification.

Authors:  Livia S A Passos; Adrien Lupieri; Dakota Becker-Greene; Elena Aikawa
Journal:  Atherosclerosis       Date:  2020-02-28       Impact factor: 5.162

10.  An Exploratory Analysis of Proprotein Convertase Subtilisin/Kexin Type 9 Inhibition and Aortic Stenosis in the FOURIER Trial.

Authors:  Brian A Bergmark; Michelle L O'Donoghue; Sabina A Murphy; Julia F Kuder; Marat V Ezhov; Richard Ceška; Ioanna Gouni-Berthold; Henrik K Jensen; S Lale Tokgozoglu; François Mach; Kurt Huber; Zbigniew Gaciong; Basil S Lewis; Francois Schiele; J Wouter Jukema; Terje R Pedersen; Robert P Giugliano; Marc S Sabatine
Journal:  JAMA Cardiol       Date:  2020-06-01       Impact factor: 14.676

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