NUFIP1

Chr 13

nuclear FMR1 interacting protein 1

Also known as: NUFIP, Rsa1, bA540M5.1

This nuclear RNA binding protein interacts with fragile X mental retardation protein (FMRP), regulates RNA polymerase II transcription, and participates in snoRNP biogenesis. Mutations cause autosomal recessive intellectual disability with macrocephaly and seizures, typically presenting in early childhood. The gene shows minimal constraint against loss-of-function variants in population databases.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.97
Clinical SummaryNUFIP1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.97LOEUF
pLI 0.000
Z-score 1.66
OE 0.63 (0.420.97)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.17Z-score
OE missense 1.03 (0.931.14)
268 obs / 260.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.63 (0.420.97)
00.351.4
Missense OE1.03 (0.931.14)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 15 / 23.7Missense obs/exp: 268 / 260.4Syn Z: -1.24
DN
0.6356th %ile
GOF
0.3094th %ile
LOF
0.4726th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NUFIP1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found