NBPF12

Chr 1

NBPF member 12

Also known as: COAS1, KIAA1245

The NBPF12 protein contains tandemly repeated DUF1220 domains and is part of the neuroblastoma breakpoint gene family that has undergone extensive duplication in humans. Copy number variations in the chromosomal region containing NBPF genes have been associated with multiple neurodevelopmental conditions including microcephaly, macrocephaly, autism, schizophrenia, and cognitive disability, as well as congenital heart disease and kidney anomalies. This gene shows extremely low constraint against loss-of-function variants, suggesting tolerance to complete gene loss.

OMIMResearchSummary from RefSeq
MultiplemechanismLOEUF 1.83
Clinical SummaryNBPF12
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
217 unique Pathogenic / Likely Pathogenic· 6 VUS of 234 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.83LOEUF
pLI 0.000
Z-score -1.14
OE 1.32 (0.921.83)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-5.43Z-score
OE missense 2.18 (1.882.00)
366 obs / 167.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.32 (0.921.83)
00.351.4
Missense OE2.18 (1.882.00)
00.61.4
Synonymous OE1.74
01.21.6
LoF obs/exp: 20 / 15.2Missense obs/exp: 366 / 167.9Syn Z: -4.77
DN
0.6261th %ile
GOF
0.88top 5%
LOF
0.3745th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

234 submitted variants in ClinVar

Classification Summary

Pathogenic192
Likely Pathogenic25
VUS6
Likely Benign6
Benign1
Conflicting1
192
Pathogenic
25
Likely Pathogenic
6
VUS
6
Likely Benign
1
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
192
Likely Pathogenic
25
VUS
6
Likely Benign
6
Benign
1
Conflicting
1
Total231

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

NBPF12 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found