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F350I

Category 3/4 — Most DruggableLikely pathogenicTransmembrane · predictedEditorial
PhenylalanineIsoleucine at position 350 · TM2 (340-360), helical transmembrane · WFS1 (Wolframin)

Phenylalanine → Isoleucine at position 350 inside TM2. ClinVar Likely pathogenic. AlphaMissense 0.934, DynaMut2 ΔΔG +0.52 kcal/mol — STABILISING. Aromatic-to-branched-aliphatic substitution in a TM helix.

Interactive 3D Structure

Wild-type reference
Wild-type F350 — hydrogen bond to F354
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DynaMut2 mutant · F350I
Mutant I350 — polar contact to P346 lost (5 contacts lost)
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Bond changes · DynaMut2 interaction analysis

5 lost2 gained14 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondP346P346Preserved
Hydrogen bondL347L347Preserved
Hydrogen bondS353S353Preserved
Hydrogen bondF354F354Preserved
Hydrogen bondS418Gained
Polar contactP346P346Preserved
Polar contactL347L347Preserved
Polar contactV348V348Preserved
Polar contactL352L352Preserved
Polar contactS353S353Preserved
Polar contactF354F354Preserved
Polar contactS418S418Preserved
Aromatic / πF419Lost
Van der WaalsV348V348Preserved
Van der WaalsF354F354Preserved
Van der WaalsS418Lost
HydrophobicV415Lost
HydrophobicF419Lost
HydrophobicA422Gained
HydrophobicI427I427Preserved
HydrophobicL432Lost

Lost / gained / preserved interatomic contacts at the variant residue, from the DynaMut2 (Arpeggio) interaction analysis of the wild-type and energy-minimized mutant structures.

Computational Predictions

DynaMut2 ΔΔG
0.52kcal/mol
Stabilising — mild
AlphaMissense
0.934
LPath
AlphaFold pLDDT
93
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable
ΔΔG confidence: Reduced confidence · Transmembrane
  • transmembrane position; predictor benchmarked on soluble proteins

This variant sits in the transmembrane band. The stability value was computed with a predictor benchmarked on water-soluble proteins. Published benchmarks of ddG predictors on membrane proteins report correlation below 0.4. Treat the magnitude as unreliable and the sign as weak evidence only.

Do not rank this value against a variant from a different region on ΔΔG alone.

Clinical Evidence

ClinVar classificationLikely pathogenic
Review statuscriteria provided, single submitter
Associated conditions(no specific conditions catalogued)
InheritanceInheritance not specified.
Population frequency (gnomAD v4)Ultra-rare · AF 0.000068%
cDNA changec.1048T>A
ClinVar accessionVCV003718608
Last evaluated2025/07/28 00:00

Observed at very low frequency in gnomAD.

Classified for1★ unverified submission

ClinVar classifies this variant as Likely pathogenic, but does not state what it is classified for. ClinVar records the condition only as "not provided". A classification without a phenotype and an inheritance mode cannot be read as a statement about Wolfram syndrome risk or severity.

  • Phenotype scope is interpretation of submitted records. The computed values on this card (AlphaMissense, DynaMut2 ΔΔG, pLDDT) are measurements of the model, not of a patient.
  • Presentation cannot be generalised from neighbouring variants: one nucleotide over can present very differently. Structural similarity supports a mechanistic hypothesis, never a phenotype claim.

Review status: 1★ single submitter. ClinVar "conditions" is the union of all submissions; 0–1★ entries are submitted conditions, not documented phenotypes.

Population frequency
Global (all ancestries)
AF 0.000068% · 1 / 1,461,848 alleles
Homozygotes
0

Every ancestry group with observed alleles falls below the reporting threshold (min 2 alleles, min 500 sampled) — too sparse to name a highest-frequency population.

No homozygotes reported in gnomAD v4. For a recessive allele this is expected and is not, on its own, evidence either way.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
African / African American · under-sampled0.0030%1 / 33,4800

Source: gnomAD r4, retrieved 2026-08-21. Values are allele frequencies. The carrier estimate is derived from the allele frequency (Hardy-Weinberg, ~2·AF) and is an approximation, not a published figure — where a published carrier frequency exists it outranks this column and is cited on the card.

Structural Context

Position 350 sits in TM2. The AlphaFold model places F350 within 5 Å of ILE349 (2.5 Å), TYR351 (2.5 Å), SER418 (3.3 Å — TM2-TM3 cross-helix contact), SER353 (3.5 Å), and LEU347 (3.7 Å). The F350-S418 contact across helices is the structurally significant observation.

The wild-type phenylalanine's aromatic ring likely makes π-stacking or edge-face contact with Y351 and aromatic packing with the surrounding helices. Replacing it with isoleucine eliminates the aromatic character and replaces it with branched aliphatic packing. The DynaMut2 ΔΔG of +0.52 (stabilising) reflects that isoleucine packs efficiently into the local hydrophobic environment.

But AlphaMissense's 0.934 confirms severe functional consequence. The mechanism is loss of aromatic π-stacking with Y351 plus perturbation of the F350-S418 TM2-TM3 cross-helix contact.

Amino-acid chemistry
Phenylalanine (F) → Isoleucine (I) — aromatic hydrophobic replaced by branched aliphatic hydrophobic. Aromatic π-system lost; volume comparable.
Position in the protein
TM2 (residues 340–360) · position 350 mid-helix, bilayer-embedded (pLDDT 93 — high confidence).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted. ΔΔG = +0.52 stabilising. AlphaMissense 0.934 confirms severe functional consequence.

Mechanism is loss of aromatic packing with Y351 plus TM2-TM3 interface disruption at S418. Therapeutic strategy: site-directed at the F350-Y351-S418 contact cluster.

Why this matters

F350I joins the stabilising-but-pathogenic class. The TM2-TM3 cross-helix S418 contact identifies a previously-unseen TM-TM target.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the F350I PDF below and upload it to Wolfram Intelligence to generate therapeutic-strategy proposals — guanidinium mimetics, sigma-1 agonist docking, NAC thiol-capping. NAC is already on the bench-testing list.

Download F350I PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane340360 · Helical
Natural variant350350 · in WFS1