RANBP1

Chr 22

RAN binding protein 1

Also known as: HTF9A

This protein regulates nucleocytoplasmic transport by forming complexes with RAN GTPase and controlling the movement of proteins and nucleic acids between the nucleus and cytoplasm, and is required for normal mitotic spindle assembly. Mutations cause autosomal dominant neurodevelopmental disorder with microcephaly, seizures, and brain malformations. The gene is highly constrained against loss-of-function variants, indicating intolerance to disruption.

ResearchSummary from RefSeq, UniProt
LOEUF 0.47
Clinical SummaryRANBP1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.82) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
379 unique Pathogenic / Likely Pathogenic· 53 VUS of 449 total submissions
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.47LOEUF
pLI 0.822
Z-score 2.65
OE 0.10 (0.030.47)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.99Z-score
OE missense 0.49 (0.390.60)
58 obs / 119.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.10 (0.030.47)
00.351.4
Missense OE0.49 (0.390.60)
00.61.4
Synonymous OE1.20
01.21.6
LoF obs/exp: 1 / 10.1Missense obs/exp: 58 / 119.2Syn Z: -1.06

ClinVar Variant Classifications

449 submitted variants in ClinVar

Classification Summary

Pathogenic370
Likely Pathogenic9
VUS53
Likely Benign3
Benign2
370
Pathogenic
9
Likely Pathogenic
53
VUS
3
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
370
0
370
Likely Pathogenic
0
0
9
0
9
VUS
1
41
11
0
53
Likely Benign
0
1
0
2
3
Benign
0
0
1
1
2
Total1423913437

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

View in ClinVar →

Protein Context — Lollipop Plot

RANBP1 · 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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