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F264L

Category 5 — IDR ExclusionConflictingCytoplasmic · predictedEditorial
PhenylalanineLeucine at position 264 · N-terminal cytoplasmic domain (87-313) · WFS1 (Wolframin)

Phenylalanine → Leucine at position 264 in N-terminal cytoplasmic domain. ClinVar Conflicting including Wolfram-like syndrome. AlphaMissense 0.29 (below threshold) — AM under-call. DynaMut2 ΔΔG +0.19 kcal/mol (stabilising). pLDDT 47 — Category 5 IDR territory.

Interactive 3D Structure

Wild-type reference
Wild-type F264 — hydrogen bond to S261
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DynaMut2 mutant · F264L
Mutant L264 — hydrogen bond to S261 lost (2 contacts lost)
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Bond changes · DynaMut2 interaction analysis

2 lost1 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondS261S261Preserved
Polar contactS261Lost
Polar contactS262S262Preserved
Polar contactQ266Gained
HydrophobicI259Lost

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
0.19kcal/mol
Stabilising — mild
AlphaMissense
0.287
LBen
AlphaFold pLDDT
47
model confidence
Schema
Cat 5
Category 5 — IDR Exclusion

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWFS1-related disorder; Wolfram-like syndrome
InheritanceWFS1-related disorder + Wolfram-like syndrome documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0011%
cDNA changec.792C>G
ClinVar accessionVCV000504707
Last evaluated2025/06/12 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 264 sits in wolframin's N-terminal cytoplasmic domain at the boundary of the IDR. pLDDT of 47 is BELOW the 50 threshold — the Atlas formally classifies this position as Category 5 (IDR exclusion). DynaMut2 predictions in this region are not fully trustworthy.

Neighbors: LEU263 (2.5 Å), LEU265 (2.5 Å), SER261 (4.1 Å). The neighbor sparsity is itself an IDR signature.

AlphaMissense's 0.29 is below threshold. The Conflicting ClinVar classifications and the IDR localization together flag this variant as one where computational drug discovery should pause for wet-lab characterization.

Amino-acid chemistry
Phenylalanine (F) → Leucine (L) — aromatic hydrophobic replaced by branched aliphatic hydrophobic. Aromatic π-system lost.
Position in the protein
N-terminal cytoplasmic domain · position 264 in a borderline-IDR region (pLDDT 47).

Druggability Assessment

Category 5 — IDR Exclusion (borderline). pLDDT 47 below the 50 threshold. AlphaMissense 0.29 below threshold. DynaMut2 prediction not trustworthy.

The Atlas routes Category 5 variants to wet-lab characterization rather than computational drug discovery.

Why this matters

F264L is the latest Category 5 IDR-region variant in the Atlas. Wet-lab validation required before therapeutic strategy is set.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the F264L 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 F264L PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Region1321 · Interaction with ATP6V1A