FANCG

Chr 9AR

FA complementation group G

Also known as: FAG, XRCC9

The FANCG protein functions in DNA repair, specifically in interstrand DNA cross-link repair and maintenance of chromosome stability as part of the Fanconi anemia nuclear protein complex. Mutations cause Fanconi anemia complementation group G, a disorder characterized by bone marrow failure, congenital anomalies, cytogenetic instability, and increased cancer predisposition. This gene follows autosomal recessive inheritance and is highly intolerant to loss-of-function variants.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.092 OMIM phenotypes
Clinical SummaryFANCG
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Gene-Disease Validity (ClinGen)
Fanconi anemia complementation group G · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
21 unique Pathogenic / Likely Pathogenic· 269 VUS of 400 total submissions
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Clinical Trials
2 active or recruiting trials — 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
1.09LOEUF
pLI 0.000
Z-score 1.15
OE 0.78 (0.571.09)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.24Z-score
OE missense 0.96 (0.871.06)
301 obs / 313.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.78 (0.571.09)
00.351.4
Missense OE0.96 (0.871.06)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 25 / 32.0Missense obs/exp: 301 / 313.2Syn Z: 0.01

ClinVar Variant Classifications

400 submitted variants in ClinVar

Classification Summary

Pathogenic7
Likely Pathogenic14
VUS269
Likely Benign97
Conflicting1
7
Pathogenic
14
Likely Pathogenic
269
VUS
97
Likely Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
4
0
3
0
7
Likely Pathogenic
13
0
1
0
14
VUS
3
249
10
7
269
Likely Benign
0
9
15
73
97
Benign
0
0
0
0
0
Conflicting
1
Total202582980388

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

View in ClinVar →

Protein Context — Lollipop Plot

FANCG · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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