NFX1

Chr 9

nuclear transcription factor, X-box binding 1

Also known as: NFX2, TEG-42, Tex42

MHC class II gene expression is controlled primarily at the transcriptional level by transcription factors that bind to the X and Y boxes, two highly conserved elements in the proximal promoter of MHC class II genes. The protein encoded by this gene is a transcriptional repressor capable of binding to the conserved X box motif of HLA-DRA and other MHC class II genes in vitro. The protein may play a role in regulating the duration of an inflammatory response by limiting the period in which class II MHC molecules are induced by IFN-gamma. Three alternative splice variants, each of which encodes a different isoform, have been identified. [provided by RefSeq, Jul 2008]

0
Active trials
67
Pathogenic / LP
205
ClinVar variants
9
Pubs (1 yr)
1.4
Missense Z
0.46
LOEUF
Clinical SummaryNFX1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.
📋
ClinVar Variants
67 Pathogenic / Likely Pathogenic· 130 VUS of 205 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.46LOEUF
pLI 0.000
Z-score 4.99
OE 0.31 (0.220.46)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.41Z-score
OE missense 0.84 (0.780.90)
520 obs / 618.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.31 (0.220.46)
00.351.4
Missense OE0.84 (0.780.90)
00.61.4
Synonymous OE0.94
01.21.6
LoF obs/exp: 19 / 61.1Missense obs/exp: 520 / 618.8Syn Z: 0.67

ClinVar Variant Classifications

205 submitted variants in ClinVar

Classification Summary

Pathogenic59
Likely Pathogenic8
VUS130
Likely Benign8
59
Pathogenic
8
Likely Pathogenic
130
VUS
8
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
59
0
59
Likely Pathogenic
0
0
8
0
8
VUS
0
120
10
0
130
Likely Benign
0
6
0
2
8
Benign
0
0
0
0
0
Total0126772205

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

View in ClinVar →

NFX1 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Landmark / reviewRecent case evidence
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC