CALCRL

Chr 2AR

calcitonin receptor like receptor

Also known as: CGRPR, CRLR, LMPHM8

The protein functions as a G protein-coupled receptor that forms complexes with receptor-activity-modifying proteins (RAMPs) to bind calcitonin gene-related peptide and adrenomedullin, activating cAMP-dependent signaling pathways. Mutations cause lymphatic malformation with autosomal recessive inheritance. The gene is highly constrained against loss-of-function variants, indicating critical importance for normal development.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 0.301 OMIM phenotype
Clinical SummaryCALCRL
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). One damaged copy is likely sufficient to cause disease.
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Clinical Trials
4 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.30LOEUF
pLI 0.985
Z-score 4.18
OE 0.12 (0.050.30)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.09Z-score
OE missense 0.62 (0.540.71)
144 obs / 234.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.12 (0.050.30)
00.351.4
Missense OE0.62 (0.540.71)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 3 / 26.0Missense obs/exp: 144 / 234.1Syn Z: 0.63
DN
0.6649th %ile
GOF
0.77top 25%
LOF
0.3940th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function, loss-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
LOFLOEUF 0.30
DNprediction 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. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CALCRL · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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