RFC2

Chr 7

replication factor C subunit 2

Also known as: RFC40

This gene encodes the 40 kD subunit of replication factor C (RFC), a protein complex that loads proliferating cell nuclear antigen (PCNA) onto DNA during replication by DNA polymerases delta and epsilon. Biallelic mutations cause severe combined immunodeficiency with microcephaly, growth restriction, and sensitivity to DNA-damaging agents, following autosomal recessive inheritance. The gene shows low constraint against loss-of-function variants, consistent with the recessive inheritance pattern observed clinically.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.98
Clinical SummaryRFC2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
💊
Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.98LOEUF
pLI 0.000
Z-score 1.64
OE 0.60 (0.390.98)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.15Z-score
OE missense 0.77 (0.680.88)
160 obs / 206.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.60 (0.390.98)
00.351.4
Missense OE0.77 (0.680.88)
00.61.4
Synonymous OE1.06
01.21.6
LoF obs/exp: 12 / 19.9Missense obs/exp: 160 / 206.7Syn Z: -0.46
DN
0.79top 25%
GOF
0.5170th %ile
LOF
0.2774th %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

RFC2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →