DNALI1

Chr 1AR

dynein axonemal light intermediate chain 1

Also known as: P28, SPGF83, dJ423B22.5, hp28

This gene encodes a protein involved in sperm flagellum assembly and is a candidate gene for primary ciliary dyskinesia based on its homology to Chlamydomonas inner dynein arm components. Mutations cause spermatogenic failure with autosomal recessive inheritance. The gene shows minimal constraint against loss-of-function variants (very low pLI score), which is consistent with its recessive disease pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 1.511 OMIM phenotype
Clinical SummaryDNALI1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.51LOEUF
pLI 0.000
Z-score 0.01
OE 1.00 (0.681.51)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.02Z-score
OE missense 1.00 (0.881.13)
168 obs / 168.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.00 (0.681.51)
00.351.4
Missense OE1.00 (0.881.13)
00.61.4
Synonymous OE1.10
01.21.6
LoF obs/exp: 16 / 16.0Missense obs/exp: 168 / 168.6Syn Z: -0.65
DN
0.76top 25%
GOF
0.3491th %ile
LOF
0.4528th %ile

The highest-scoring mechanism for this gene is dominant-negative.

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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DNALI1 · 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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