FSAF1

Chr 1

40S small subunit processome assembly factor 1

Also known as: C1orf131

FSAF1 encodes a protein that is part of the small subunit processome involved in ribosome biogenesis, where it helps coordinate the assembly and processing of ribosomal RNA precursors in the nucleolus. Mutations cause autosomal recessive neurodevelopmental disorder with microcephaly, seizures, and brain malformations. This condition presents in early infancy with severe developmental delays and progressive neurological deterioration.

ResearchSummary from UniProt
Clinical SummaryFSAF1
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ClinVar Variants
38 unique Pathogenic / Likely Pathogenic· 11 VUS of 66 total submissions
Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

66 submitted variants in ClinVar

Classification Summary

Pathogenic36
Likely Pathogenic2
VUS11
Likely Benign2
Benign1
36
Pathogenic
2
Likely Pathogenic
11
VUS
2
Likely Benign
1
Benign

Curated Variants Distribution

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

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
36
Likely Pathogenic
2
VUS
11
Likely Benign
2
Benign
1
Total52

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

FSAF1 · 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
Open Research Assistant →

No publications found for FSAF1