NFRKB

Chr 11

nuclear factor related to kappaB binding protein

Also known as: INO80G

The protein binds to specific DNA sequences and functions in chromatin remodeling and regulation of chromosome organization as part of the Ino80 complex. NFRKB mutations cause autosomal recessive neurodevelopmental disorders with intellectual disability and developmental delay. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.368), suggesting some intolerance to complete protein loss.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
78
P/LP submissions
0%
P/LP missense
0.37
LOEUF
Mechanism
Clinical SummaryNFRKB
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.24) despite low pLI — interpret in context.
📋
ClinVar Variants
78 unique Pathogenic / Likely Pathogenic· 166 VUS of 282 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.37LOEUF
pLI 0.134
Z-score 5.58
OE 0.24 (0.160.37)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.53Z-score
OE missense 0.85 (0.790.90)
659 obs / 779.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.24 (0.160.37)
00.351.4
Missense OE0.85 (0.790.90)
00.61.4
Synonymous OE1.12
01.21.6
LoF obs/exp: 15 / 62.7Missense obs/exp: 659 / 779.5Syn Z: -1.66

ClinVar Variant Classifications

282 submitted variants in ClinVar

Classification Summary

Pathogenic73
Likely Pathogenic5
VUS166
Likely Benign9
Conflicting1
73
Pathogenic
5
Likely Pathogenic
166
VUS
9
Likely Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
73
0
73
Likely Pathogenic
0
0
5
0
5
VUS
0
166
0
0
166
Likely Benign
0
6
0
3
9
Benign
0
0
0
0
0
Conflicting
1
Total0172783254

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

View in ClinVar →

Protein Context — Lollipop Plot

NFRKB · protein map & ClinVar variants

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

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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