CENPN

Chr 16

centromere protein N

Also known as: BM039, C16orf60, CENP-N, ICEN32

The protein forms part of the nucleosome-associated complex that is essential for kinetochore assembly and chromosome segregation by directly binding to CENPA nucleosomes at centromeres. Mutations cause autosomal recessive primary microcephaly with growth retardation and developmental delays. This gene is moderately constrained against loss-of-function variants, and the condition typically presents in early infancy with severe microcephaly and failure to thrive.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.65
Clinical SummaryCENPN
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
41 unique Pathogenic / Likely Pathogenic· 81 VUS of 157 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.65LOEUF
pLI 0.004
Z-score 2.81
OE 0.36 (0.210.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.43Z-score
OE missense 1.09 (0.971.22)
202 obs / 185.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.36 (0.210.65)
00.351.4
Missense OE1.09 (0.971.22)
00.61.4
Synonymous OE1.21
01.21.6
LoF obs/exp: 8 / 22.3Missense obs/exp: 202 / 185.7Syn Z: -1.36

ClinVar Variant Classifications

157 submitted variants in ClinVar

Classification Summary

Pathogenic36
Likely Pathogenic5
VUS81
Likely Benign5
Benign1
36
Pathogenic
5
Likely Pathogenic
81
VUS
5
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
36
0
36
Likely Pathogenic
0
0
5
0
5
VUS
0
54
27
0
81
Likely Benign
0
4
1
0
5
Benign
0
0
1
0
1
Total058700128

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

View in ClinVar →

Protein Context — Lollipop Plot

CENPN · 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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