FAT2

Chr 5AD

FAT atypical cadherin 2

Also known as: CDHF8, CDHR9, HFAT2, MEGF1, SCA45

This protein functions as a cell adhesion molecule that regulates cell migration and mediates organization of parallel fibers during cerebellar development. Mutations cause spinocerebellar ataxia 45, a movement disorder primarily affecting the cerebellum, inherited in an autosomal dominant pattern. The gene is highly constrained against loss-of-function variants, indicating it is intolerant to protein-truncating mutations.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismADLOEUF 0.511 OMIM phenotype
Clinical SummaryFAT2
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

Moderate LoF intolerance
LoF Constraint
0.51LOEUF
pLI 0.000
Z-score 6.38
OE 0.40 (0.320.51)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.72Z-score
OE missense 0.96 (0.930.99)
2292 obs / 2390.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.40 (0.320.51)
00.351.4
Missense OE0.96 (0.930.99)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 54 / 133.6Missense obs/exp: 2292 / 2390.6Syn Z: -1.00
DN
0.7035th %ile
GOF
0.6638th %ile
LOF
0.3454th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction 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

FAT2 · 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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