DYNC1LI1

Chr 3

dynein cytoplasmic 1 light intermediate chain 1

Also known as: DLC-A, DLIC-1, DNCLI1, LIC1

The encoded protein is a light intermediate chain subunit of the cytoplasmic dynein 1 complex that facilitates retrograde transport of vesicles and organelles along microtubules and is required for spindle assembly checkpoint progression during mitosis. Mutations cause autosomal dominant intellectual disability with neurologic and ophthalmologic features. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.33
Clinical SummaryDYNC1LI1
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.96). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
17 unique Pathogenic / Likely Pathogenic· 64 VUS of 102 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.33LOEUF
pLI 0.963
Z-score 4.20
OE 0.14 (0.070.33)
Highly constrained

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

Missense Constraint
1.04Z-score
OE missense 0.82 (0.730.92)
221 obs / 269.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.14 (0.070.33)
00.351.4
Missense OE0.82 (0.730.92)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 4 / 27.9Missense obs/exp: 221 / 269.0Syn Z: 0.66
DN
0.4388th %ile
GOF
0.4382th %ile
LOF
0.69top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.33

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

102 submitted variants in ClinVar

Classification Summary

Pathogenic16
Likely Pathogenic1
VUS64
Likely Benign1
16
Pathogenic
1
Likely Pathogenic
64
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
16
0
16
Likely Pathogenic
0
0
1
0
1
VUS
0
62
2
0
64
Likely Benign
0
1
0
0
1
Benign
0
0
0
0
0
Total06319082

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

View in ClinVar →

Protein Context — Lollipop Plot

DYNC1LI1 · 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
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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC