FANCM

Chr 14AR

FA complementation group M

Also known as: FAAP250, KIAA1596, POF15, SPGF28

FANCM encodes a DNA-dependent ATPase that is essential for the Fanconi anemia DNA repair pathway, processing branched DNA structures and enabling cellular resistance to DNA crosslinking damage. Biallelic mutations cause premature ovarian failure and male spermatogenic failure with autosomal recessive inheritance, representing a milder phenotype than classical Fanconi anemia. The gene is highly constrained against loss-of-function variants (LOEUF 0.59), indicating strong selection pressure.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.592 OMIM phenotypes
Clinical SummaryFANCM
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Gene-Disease Validity (ClinGen)
hereditary breast carcinoma · ADLimited

Limited evidence — not for standalone diagnostic reporting

3 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
12 unique Pathogenic / Likely Pathogenic· 317 VUS of 500 total submissions
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — FANCM
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.59LOEUF
pLI 0.000
Z-score 4.73
OE 0.46 (0.350.59)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
-0.13Z-score
OE missense 1.01 (0.961.06)
1053 obs / 1041.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.46 (0.350.59)
00.351.4
Missense OE1.01 (0.961.06)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 40 / 87.9Missense obs/exp: 1053 / 1041.0Syn Z: -0.00
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongFANCM-related Fanconi anemiaLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6647th %ile
GOF
0.2497th %ile
LOF
0.3941th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

500 submitted variants in ClinVar

Classification Summary

Pathogenic9
Likely Pathogenic3
VUS317
Likely Benign147
Benign1
Conflicting1
9
Pathogenic
3
Likely Pathogenic
317
VUS
147
Likely Benign
1
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
9
0
0
0
9
Likely Pathogenic
3
0
0
0
3
VUS
0
306
7
4
317
Likely Benign
0
12
13
122
147
Benign
0
0
1
0
1
Conflicting
1
Total1231821126478

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

View in ClinVar →

Protein Context — Lollipop Plot

FANCM · 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 →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗