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E431Q

Category 3/4 — Most DruggableLikely pathogenicTransmembrane · predictedEditorial
GlutamateGlutamine at position 431 · TM4 (427-447), helical transmembrane · WFS1 (Wolframin)

Glutamate → Glutamine at position 431 inside TM4. ClinVar Likely pathogenic for Wolfram syndrome 1. AlphaMissense 0.959, DynaMut2 ΔΔG -0.87 kcal/mol (destabilising). The fifth Atlas variant directly involving E431 — the lumenal-membrane hub residue.

Interactive 3D Structure

Wild-type reference
Wild-type E431 — ionic bond to R558
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DynaMut2 mutant · E431Q
Mutant Q431 — ionic bond to R558 lost (9 contacts lost)
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Bond changes · DynaMut2 interaction analysis

9 lost0 gained12 preserved
Interaction typeWild-type partnerMutant partnerStatus
Ionic bondR558Lost
Hydrogen bondP428P428Preserved
Hydrogen bondV434V434Preserved
Hydrogen bondI435I435Preserved
Hydrogen bondR558Lost
Hydrogen bondY563Y563Preserved
Polar contactP428P428Preserved
Polar contactC429C429Preserved
Polar contactA433Lost
Polar contactV434V434Preserved
Polar contactI435I435Preserved
Polar contactR558Lost
Polar contactA559Lost
Polar contactY563Y563Preserved
Van der WaalsC429Lost
Van der WaalsA433Lost
Van der WaalsI435Lost
Van der WaalsA559Lost
HydrophobicP428P428Preserved
HydrophobicA559A559Preserved
HydrophobicY563Y563Preserved

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
-0.87kcal/mol
Destabilising — mild
AlphaMissense
0.959
LPath
AlphaFold pLDDT
90
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationLikely pathogenic
Review statuscriteria provided, single submitter
Associated conditionsWolfram syndrome 1
InheritanceWolfram syndrome 1 (AR) documented.
Population frequency (gnomAD v4)Absent from gnomAD v4
cDNA changec.1291G>C
ClinVar accessionVCV001027490
Last evaluated2021/01/04 00:00

Not observed in ~730k individuals — consistent with a rare allele (ACMG PM2_supporting).

Structural Context

Position 431 is the E431 hub residue itself. The AlphaFold model places E431 within 5 Å of LEU432 (2.4 Å), SER430 (2.5 Å — partner of S430W Atlas card), PRO428 (3.6 Å — partner of P428R Atlas card), ALA559 (3.9 Å — partner of A559D Atlas card), and TYR563 (4.0 Å). The neighbor list reads like a roll call of pathogenic Atlas variants — E431 is in spatial contact with the substituted positions in four other Atlas cards.

Replacing E431 with glutamine eliminates the negative charge at this hub position. The salt bridges and electrostatic contacts that E431 maintained with R558 (across the loop) and with S430's hydroxyl and the lumenal interface lose their negative anchor.

The |ΔΔG| of 0.87 reflects substantial fold cost — E431's role is structurally central. AlphaMissense 0.959 + Wolfram syndrome 1 confirm severe functional consequence.

Amino-acid chemistry
Glutamate (E) → Glutamine (Q) — negatively-charged carboxylate replaced by neutral polar amide. Loss of charge; H-bonding preserved.
Position in the protein
TM4 (residues 427–447) · position 431 near the lumenal end of TM4 (pLDDT 90).

Druggability Assessment

Category 3/4 — Most Druggable (HUB residue). |ΔΔG| = 0.87 — fold survives at meaningful cost. AlphaMissense 0.959 + Wolfram 1 confirm severe functional consequence.

E431 is the connective hub residue at the lumenal-TM4-connecting loop boundary. The Atlas now contains 5 variants involving E431 (E431Q this card, A559D, P428R, S430W, plus R558C/R558H/A559D microregion). Drug discovery targeting E431 has multi-variant convergence.

Why this matters

E431Q is the variant AT the hub position — where four other Atlas variants contact in their neighbor analyses. Drug discovery targeting the E431 microregion is the highest-leverage docking site in the WFS1 lumenal-membrane interface region.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the E431Q 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 E431Q PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane427447 · Helical