FANK1

Chr 10

fibronectin type III and ankyrin repeat domains 1

Also known as: HSD13

FANK1 encodes a protein that regulates apoptosis through activation of JUN and AP-1-mediated transcription. Mutations cause autosomal recessive intellectual disability with seizures and corpus callosum abnormalities. The gene is not highly constrained against loss-of-function variants, consistent with recessive inheritance patterns.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.18
Clinical SummaryFANK1
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
1.18LOEUF
pLI 0.000
Z-score 0.94
OE 0.78 (0.531.18)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.55Z-score
OE missense 0.89 (0.781.01)
173 obs / 194.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.78 (0.531.18)
00.351.4
Missense OE0.89 (0.781.01)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 16 / 20.6Missense obs/exp: 173 / 194.8Syn Z: 0.75
DN
0.6453th %ile
GOF
0.6053th %ile
LOF
0.3843th %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

FANK1 · 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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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗
Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗