DNAI1

Chr 9

dynein axonemal intermediate chain 1

Also known as: CILD1, DIC1, ICS1, PCD, oda6

This gene encodes a member of the dynein intermediate chain family. The encoded protein is part of the dynein complex in respiratory cilia. The inner- and outer-arm dyneins, which bridge between the doublet microtubules in axonemes, are the force-generating proteins responsible for the sliding movement in axonemes. The intermediate and light chains, thought to form the base of the dynein arm, help mediate attachment and may also participate in regulating dynein activity. Mutations in this gene result in abnormal ciliary ultrastructure and function associated with primary ciliary dyskinesia and Kartagener syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCiliary dyskinesia, primary, 1
UniProtKartagener syndrome

Clinical highlights

Gene-disease validity (ClinGen)
primary ciliary dyskinesia 1 · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
10
Pubs (1 yr)
P/LP submissions
P/LP missense
0.72
LOEUF
DN
Mechanism· predicted
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GeneReview available — DNAI1
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.72LOEUF
pLI 0.000
Z-score 2.91
OE 0.49 (0.340.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
-0.29Z-score
OE missense 1.04 (0.961.14)
382 obs / 366.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.49 (0.340.72)
00.351.4
Missense OE?1.04 (0.961.14)
00.61.4
Synonymous OE?1.17
01.21.6
LoF obs/exp: 19 / 38.4Missense obs/exp: 382 / 366.2Syn Z: -1.64

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DNAI1 · protein map & ClinVar variants

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