DNAI7

Chr 12

dynein axonemal intermediate chain 7

Also known as: CASC1, CFAP94, LAS1, PPP1R54

This protein is predicted to bind beta-tubulin and microtubules as part of the axonemal dynein complex that regulates cilia function and may also regulate the cell cycle. Mutations in DNAI7 cause primary ciliary dyskinesia, a disorder affecting the respiratory system, fertility, and organ positioning that follows autosomal recessive inheritance. The gene shows no intolerance to loss-of-function variants in the general population.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
1
Pubs (1 yr)
38
P/LP submissions
0%
P/LP missense
0.96
LOEUF
Mechanism
Clinical SummaryDNAI7
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
38 unique Pathogenic / Likely Pathogenic· 106 VUS of 168 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.96LOEUF
pLI 0.000
Z-score 1.77
OE 0.69 (0.510.96)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.83Z-score
OE missense 0.88 (0.800.96)
307 obs / 350.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.69 (0.510.96)
00.351.4
Missense OE0.88 (0.800.96)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 27 / 38.9Missense obs/exp: 307 / 350.5Syn Z: -0.03

ClinVar Variant Classifications

168 submitted variants in ClinVar

Classification Summary

Pathogenic36
Likely Pathogenic2
VUS106
Likely Benign14
36
Pathogenic
2
Likely Pathogenic
106
VUS
14
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
36
0
36
Likely Pathogenic
0
0
2
0
2
VUS
0
99
7
0
106
Likely Benign
0
7
6
1
14
Benign
0
0
0
0
0
Total0106511158

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

View in ClinVar →

Protein Context — Lollipop Plot

DNAI7 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found