RCL1

Chr 9

RNA terminal phosphate cyclase like 1

Also known as: RNAC, RPCL1

The RCL1 protein functions as an RNA endonuclease that is essential for ribosomal small subunit biogenesis, specifically in the early pre-rRNA processing steps required for proper 18S RNA maturation. Mutations cause autosomal recessive developmental delay with variable intellectual disability and dysmorphic features. This gene shows moderate constraint against loss-of-function variants, suggesting that complete loss of function is not well-tolerated during development.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.57
Clinical SummaryRCL1
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.57LOEUF
pLI 0.059
Z-score 2.98
OE 0.29 (0.160.57)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
-1.05Z-score
OE missense 1.20 (1.081.33)
258 obs / 214.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.29 (0.160.57)
00.351.4
Missense OE1.20 (1.081.33)
00.61.4
Synonymous OE1.29
01.21.6
LoF obs/exp: 6 / 20.6Missense obs/exp: 258 / 214.7Syn Z: -2.08

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RCL1 · 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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