FANCL

Chr 2AR

FA complementation group L

Also known as: FAAP43, PHF9, POG

This protein functions as a ubiquitin ligase that mediates monoubiquitination of FANCD2 and FANCI, which are key steps in the DNA damage repair pathway. Mutations cause Fanconi anemia complementation group L, a disorder characterized by cytogenetic instability, hypersensitivity to DNA crosslinking agents, increased chromosomal breakage, and defective DNA repair. The condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.612 OMIM phenotypes
Clinical SummaryFANCL
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Gene-Disease Validity (ClinGen)
Fanconi anemia complementation group L · 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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Clinical Trials
5 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
1.61LOEUF
pLI 0.000
Z-score -0.93
OE 1.20 (0.901.61)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-1.31Z-score
OE missense 1.26 (1.141.40)
251 obs / 199.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.20 (0.901.61)
00.351.4
Missense OE1.26 (1.141.40)
00.61.4
Synonymous OE1.09
01.21.6
LoF obs/exp: 31 / 25.9Missense obs/exp: 251 / 199.0Syn Z: -0.60

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FANCL · 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

Clinical Literature
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