CIMAP2

Chr 1

ciliary microtubule associated protein 2

Also known as: C1orf177, LEM, LEXM

This gene encodes a ciliary and flagellar associated protein that localizes to the ciliary axoneme and is involved in ciliary motility. Mutations cause primary ciliary dyskinesia-40, an autosomal recessive disorder characterized by chronic respiratory tract infections, bronchiectasis, and situs inversus in approximately half of patients. The gene shows relatively low constraint against loss-of-function variants.

LOEUF 1.28
Clinical SummaryCIMAP2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
11 unique Pathogenic / Likely Pathogenic· 65 VUS of 82 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.28LOEUF
pLI 0.000
Z-score 0.49
OE 0.89 (0.631.28)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.20Z-score
OE missense 0.96 (0.861.08)
228 obs / 236.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.89 (0.631.28)
00.351.4
Missense OE0.96 (0.861.08)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 21 / 23.5Missense obs/exp: 228 / 236.5Syn Z: 0.69

ClinVar Variant Classifications

82 submitted variants in ClinVar

Classification Summary

Pathogenic8
Likely Pathogenic3
VUS65
Likely Benign2
8
Pathogenic
3
Likely Pathogenic
65
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
8
0
8
Likely Pathogenic
0
0
3
0
3
VUS
0
60
5
0
65
Likely Benign
0
1
0
1
2
Benign
0
0
0
0
0
Total06116178

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

CIMAP2 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found