CDK5

Chr 7AR

cyclin dependent kinase 5

Also known as: LIS7, PSSALRE

This gene encodes a proline-directed serine/threonine kinase that functions in neuronal migration, synaptic plasticity, and cytoskeletal organization by phosphorylating key proteins involved in these processes. Mutations cause autosomal recessive lissencephaly-7 with cerebellar hypoplasia, a severe brain malformation disorder. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.562) and has high expression in postmitotic central nervous system neurons.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.561 OMIM phenotype
Clinical SummaryCDK5
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Gene-Disease Validity (ClinGen)
lissencephaly with cerebellar hypoplasia · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.27) despite low pLI — interpret in context.
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ClinVar Variants
76 unique Pathogenic / Likely Pathogenic· 21 VUS of 171 total submissions
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GeneReview available — CDK5
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.56LOEUF
pLI 0.139
Z-score 2.94
OE 0.27 (0.140.56)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
3.07Z-score
OE missense 0.33 (0.270.42)
56 obs / 167.8 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.27 (0.140.56)
00.351.4
Missense OE0.33 (0.270.42)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 5 / 18.7Missense obs/exp: 56 / 167.8Syn Z: 0.03
DN
0.6936th %ile
GOF
0.6052th %ile
LOF
0.3647th %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

171 submitted variants in ClinVar

Classification Summary

Pathogenic71
Likely Pathogenic5
VUS21
Likely Benign48
Benign11
Conflicting1
71
Pathogenic
5
Likely Pathogenic
21
VUS
48
Likely Benign
11
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
0
70
0
71
Likely Pathogenic
0
1
4
0
5
VUS
0
12
9
0
21
Likely Benign
0
0
20
28
48
Benign
0
0
10
1
11
Conflicting
1
Total11311329157

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

View in ClinVar →

Protein Context — Lollipop Plot

CDK5 · 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
Open Research Assistant →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗