Gene Information

PCSK9

proprotein convertase subtilisin/kexin type 9 [Source:HGNC Symbol;Acc:HGNC:20001]

protein_codingChromosome 1ENSG00000169174

Overview

PCSK9 variants are associated with 1 disease including homozygous familial hypercholesterolemia, with 6 documented clinical phenotypes.


Associated Diseases

The PCSK9 gene is associated with 1 medical condition, including:

homozygous familial hypercholesterolemia

MONDO:0018328


Clinical Phenotypes

Research has identified 6 phenotypic features associated with PCSK9 variants:

Corneal arcus

HP:0001084

Autosomal dominant inheritance

HP:0000006

Hypercholesterolemia

HP:0003124

Xanthelasma

HP:0001114

Tendon xanthomatosis

HP:0010874

Abnormal LDL cholesterol concentration

HP:0031886


ClinVar Variants

ClinVar contains 10 variants associated with PCSK9. Below are some notable variants:

NM_174936.4(PCSK9):c.399+1G>A

Likely pathogenic

VCV004277664

Review: criteria provided, single submitter

NM_174936.4(PCSK9):c.1864-651G>T

Uncertain significance

VCV004079509

Review: criteria provided, single submitter

NM_174936.4(PCSK9):c.1193T>C (p.Met398Thr)

Uncertain significance

VCV004077296

Review: criteria provided, single submitter

NM_174936.4(PCSK9):c.749T>G (p.Leu250Arg)

Uncertain significance

VCV004057032

Review: criteria provided, single submitter

NM_174936.4(PCSK9):c.874G>A (p.Gly292Arg)

Uncertain significance

VCV004055944

Review: criteria provided, single submitter


Recent Publications

There are 4,081 publications about PCSK9 indexed in PubMed. Below are some recent papers:

Impact of PCSK9 Inhibitors on Lipoprotein(a) Levels: A Multi-Center Study.

Bhatia HS, Cuomo R, Ramsis M

PMID: 41253688

2025 Nov 18

Eur J Prev Cardiol

Cell-dependent contributions of thrombospondin-1 to the rupture of abdominal aortic aneurysm in mice.

Zhou T, Yang H, Kim J

PMID: 41252281

2025 Nov 18

Cardiovasc Res

Lipid-lowering therapy in Switzerland: time trends in utilization and cost, 2015-2023.

Wolf S, Zufferey J, Kucher N

PMID: 41250151

2025 Nov 17

Lipids Health Dis

Dramatic coronary artery aneurysm regression after coronary artery bypass grafting and proprotein convertase subtilisin/kexin type 9 inhibitor therapy.

Yokoyama Y, Higashino H, Fujieda H

PMID: 41247317

2025 Nov 17

Interdiscip Cardiovasc Thorac Surg

Advances in molecular regulation and function of LDLR family in viral infection.

Yao Q, Gong J, Lu H

PMID: 41244697

2025

Front Microbiol

View all 4,081 publications on PubMed →

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Trusted Data Sources

EnsemblMonarch InitiativeClinVarGeneReviewsPubMed

Last updated: 11/20/2025

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