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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

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.

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