Gene Information

LDLR

low density lipoprotein receptor [Source:HGNC Symbol;Acc:HGNC:6547]

protein_codingChromosome 19ENSG00000130164

Overview

LDLR variants are associated with 1 disease including homozygous familial hypercholesterolemia, with 7 documented clinical phenotypes.


Associated Diseases

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

homozygous familial hypercholesterolemia

MONDO:0018328


Clinical Phenotypes

Research has identified 7 phenotypic features associated with LDLR variants:

Increased LDL cholesterol concentration

HP:0003141

Autosomal recessive inheritance

HP:0000007

Coronary artery atherosclerosis

HP:0001677

Xanthelasma

HP:0001114

Corneal arcus

HP:0001084

Autosomal dominant inheritance

HP:0000006

Tendon xanthomatosis

HP:0010874


ClinVar Variants

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

NM_000527.5(LDLR):c.68-2A>C

Pathogenic

VCV004528366

Review: criteria provided, single submitter

NM_000527.5(LDLR):c.1487del (p.Gly496fs)

Pathogenic

VCV004526913

Review: criteria provided, single submitter

NM_000527.5(LDLR):c.1187-294_1845+1200del

Pathogenic

VCV004526720

Review: criteria provided, single submitter

NM_000527.5(LDLR):c.2248del (p.Ser750fs)

Likely pathogenic

VCV004526682

Review: no assertion criteria provided

NM_000527.5(LDLR):c.2422C>T (p.Leu808Phe)

Uncertain significance

VCV004526519

Review: criteria provided, single submitter


Recent Publications

There are 2,700 publications about LDLR indexed in PubMed. Below are some recent papers:

Allogeneic hematopoietic cell transplantation initiates atherosclerosis in mice via CD8+ T cells.

Jorgacevic I, Shaikh H, Ali H

PMID: 41252283

2025 Nov 18

Cardiovasc Res

Clonal hematopoiesis activates pro-calcific pathways in macrophages and promotes aortic valve stenosis.

Abplanalp WT, Raddatz MA, Schuhmacher B

PMID: 41252200

2025 Nov 18

J Clin Invest

284 nm UVB Phototherapy Regulates Immunoinflammatory Responses and Improves Atherosclerotic Plaque Stability in Mice.

Ito K, Sasaki N, Krisnanda A

PMID: 41249043

2025 Oct 3

Kobe J Med Sci

REGULATION OF HDL DYSFUNCTIONALITY BY PHOSPHATIDYLETHANOLAMINE LINKS POLY-UNSATURATED FATTY ACIDS WITH ATHEROSCLEROTIC CARDIOVASCULAR DISEASES.

Taradeh M, Hardy LM, Dahik VD

PMID: 41248752

2025 Nov 15

Mol Metab

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 2,700 publications on PubMed →

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Trusted Data Sources

EnsemblMonarch InitiativeClinVarGeneReviewsPubMed

Last updated: 11/20/2025

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