LRRK1

Chr 15AR

leucine rich repeat kinase 1

The protein is a serine/threonine kinase that phosphorylates RAB proteins involved in intracellular trafficking and negatively regulates bone mass through osteoclast maturation. Autosomal recessive mutations cause osteosclerotic metaphyseal dysplasia, a bone disorder characterized by increased bone density and abnormal bone development. The gene is highly constrained against loss-of-function variants (LOEUF 0.493), indicating that complete loss of protein function is likely pathogenic.

OMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismARLOEUF 0.491 OMIM phenotype
Clinical SummaryLRRK1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.49LOEUF
pLI 0.000
Z-score 5.54
OE 0.37 (0.280.49)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.72Z-score
OE missense 0.78 (0.740.82)
931 obs / 1195.3 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.37 (0.280.49)
00.351.4
Missense OE0.78 (0.740.82)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 33 / 89.5Missense obs/exp: 931 / 1195.3Syn Z: -1.04
DN
0.6065th %ile
GOF
0.6932th %ile
LOF
0.3357th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LRRK1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC