FLII

Chr 17AR

FLII actin remodeling protein

Also known as: CMD2J, FLI, FLIL, Fli1

FLII encodes a protein that regulates actin polymerization and is required for proper myofibril organization and sarcomeric thin filament length regulation in cardiac muscle. Mutations cause autosomal recessive dilated cardiomyopathy (type 2J). The gene is highly constrained against loss-of-function variants (LOEUF 0.607), suggesting complete loss of protein function is likely pathogenic.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 0.611 OMIM phenotype
Clinical SummaryFLII
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Gene-Disease Validity (ClinGen)
cardiomyopathy, dilated, 2j · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.61LOEUF
pLI 0.000
Z-score 4.23
OE 0.45 (0.340.61)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.51Z-score
OE missense 0.95 (0.891.01)
753 obs / 793.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.45 (0.340.61)
00.351.4
Missense OE0.95 (0.891.01)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 31 / 68.9Missense obs/exp: 753 / 793.2Syn Z: -1.97

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FLII · 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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