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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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LRRC55
leucine rich repeat containing 55
Chromosome 11 · 11q12.1
NCBI Gene: 219527Ensembl: ENSG00000183908.7HGNC: HGNC:32324UniProt: A0A494C0H1
11PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Ion ChannelTransporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
voltage-gated potassium channel activitytransmembrane transporter bindingpotassium channel activator activitypotassium ion transmembrane transportvenous thromboembolismtype 2 diabetes mellitusfocal segmental glomerulosclerosisnephrotic syndrome
✦AI Summary

LRRC55 (leucine-rich repeat containing 55) functions as an auxiliary protein that modulates large-conductance, voltage and calcium-activated potassium (BK) channels. It produces a marked ~50 mV hyperpolarizing shift in the BK channel's voltage dependence of activation in the absence of calcium, positioning it within a γ family of BK channel auxiliary proteins distinct from classical β-subunits 1. LRRC55 shows tissue-specific expression predominantly in brain 1. In pathological contexts, upregulated LRRC55 promotes BK channel activation, leading to potassium loss and increased intracellular calcium, which triggers apoptosome formation and caspase-3 activation 2. Elevated LRRC55 expression occurs in podocytes from patients with focal segmental glomerulosclerosis, diabetic nephropathy, and membranous nephropathy 2. Genetic variants in LRRC55 (rs717117) associate with reduced anti-TNF drug response in rheumatoid arthritis patients, particularly those who are rheumatoid factor-positive, via mechanisms involving altered IL-6 production 3. Additionally, a common variant near the LRRC55 locus (rs11604583) associates with increased deep vein thrombosis risk 4. Beyond ion channel regulation, a cytoplasmic lncRNA (lncLrrc55-AS) antisense to LRRC55 enhances antiviral innate immune responses by promoting IRF3 phosphorylation 5.

Sources cited
1
LRRC55 is a BK channel auxiliary subunit producing ~50 mV hyperpolarizing shift in voltage dependence and shows tissue-specific expression in brain
PMID: 22547800
2
Upregulated LRRC55 promotes BK channel activation causing potassium loss and apoptosis in podocytes of patients with FSGS, DN, and MN
PMID: 33346797
3
LRRC55 rs717117 variant associates with reduced anti-TNF drug response in RF-positive RA patients through altered IL-6 production
PMID: 34777329
4
LRRC55 region variant (rs11604583) associates with increased deep vein thrombosis risk
PMID: 37331118
5
LncLrrc55-AS antisense RNA promotes antiviral innate immune responses by enhancing IRF3 phosphorylation
PMID: 31213650
Disease Associationsⓘ20
venous thromboembolismOpen Targets
0.19Weak
type 2 diabetes mellitusOpen Targets
0.12Weak
focal segmental glomerulosclerosisOpen Targets
0.09Suggestive
nephrotic syndromeOpen Targets
0.08Suggestive
familial idiopathic steroid-resistant nephrotic syndromeOpen Targets
0.08Suggestive
thrombophiliaOpen Targets
0.06Suggestive
nephronophthisisOpen Targets
0.06Suggestive
Balkan nephropathyOpen Targets
0.06Suggestive
nephrotic syndrome, IIa 26Open Targets
0.06Suggestive
nephrotic syndrome, type 24Open Targets
0.06Suggestive
deep vein thrombosisOpen Targets
0.05Suggestive
focal segmental glomerulosclerosis 9Open Targets
0.05Suggestive
congenital membranous nephropathy due to maternal anti-neutral endopeptidase alloimmunizationOpen Targets
0.05Suggestive
nephrotic syndrome, type 20Open Targets
0.05Suggestive
kidney diseaseOpen Targets
0.05Suggestive
nephrotic syndrome, type 4Open Targets
0.05Suggestive
primary hyperoxaluria type 2Open Targets
0.05Suggestive
focal segmental glomerulosclerosis 7Open Targets
0.05Suggestive
familial juvenile hyperuricemic nephropathy type 1Open Targets
0.05Suggestive
nephrotic syndrome, type 12Open Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
KCNK10Shared pathway100%KCNK12Shared pathway100%LRRC52Shared pathway100%LRRC26Shared pathway100%LRRC38Shared pathway84%KCNMB1Protein interaction72%
Tissue Expression6 tissues
Brain
100%
Liver
37%
Heart
25%
Bone Marrow
2%
Ovary
2%
Lung
1%
Gene Interaction Network
Click a node to explore
LRRC55KCNK10KCNK12LRRC52LRRC26LRRC38KCNMB1
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q6ZSA7
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.91LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.55 [0.35–0.91]
RankingsWhere LRRC55 stands among ~20K protein-coding genes
  • #16,816of 20,598
    Most Researched11
  • #8,332of 17,882
    Most Constrained (LOEUF)0.91
Genes detectedLRRC55
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Validation of GWAS-Identified Variants for Anti-TNF Drug Response in Rheumatoid Arthritis: A Meta-Analysis of Two Large Cohorts.
PMID: 34777329
Front Immunol · 2021
1.00
2
Upregulated LRRC55 promotes BK channel activation and aggravates cell injury in podocytes.
PMID: 33346797
J Exp Med · 2021
0.90
3
Polygenic risk scores and risk stratification in deep vein thrombosis.
PMID: 37331118
Thromb Res · 2023
0.80
4
BK potassium channel modulation by leucine-rich repeat-containing proteins.
PMID: 22547800
Proc Natl Acad Sci U S A · 2012
0.70
5
Novel candidate genes putatively involved in stress fracture predisposition detected by whole-exome sequencing.
PMID: 25023003
Genet Res (Camb) · 2014
0.60