DCX

Chr XX-linked

doublecortin

The protein regulates microtubule organization and stability to direct neuronal migration during cortical development, interacting with LIS1 for proper microtubule function. Mutations cause X-linked inheritance disorders with sex-specific phenotypes: lissencephaly in males and subcortical band heterotopia (double cortex syndrome) in females, both associated with epilepsy and cognitive disability. The pathogenic mechanism involves disrupted neuronal migration and abnormal cortical layering due to impaired microtubule function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismX-linkedLOEUF 0.622 OMIM phenotypes
Clinical SummaryDCX
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Gene-Disease Validity (ClinGen)
lissencephaly spectrum disorders · XLDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.24) despite low pLI — interpret in context.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (1)

ncbi: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.62LOEUF
pLI 0.304
Z-score 2.50
OE 0.24 (0.110.62)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
2.70Z-score
OE missense 0.44 (0.360.53)
79 obs / 181.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.24 (0.110.62)
00.351.4
Missense OE0.44 (0.360.53)
00.61.4
Synonymous OE0.78
01.21.6
LoF obs/exp: 3 / 12.6Missense obs/exp: 79 / 181.5Syn Z: 1.42

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DCX · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC