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P607R

Category 3/4 — Most DruggableConflictingTransmembrane · predictedEditorial
ProlineArginine at position 607 · TM9 (589-609), helical transmembrane · WFS1 (Wolframin)

Proline → Arginine at position 607 inside TM9. ClinVar Conflicting. AlphaMissense 0.922, ΔΔG +0.03 (neutral). pLDDT 66 borderline. Proline-removal in TM9 with charge introduction.

Interactive 3D Structure

Wild-type reference
Wild-type P607 — hydrogen bond to R611
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DynaMut2 mutant · P607R
Mutant R607 — hydrogen bond to C604 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost0 gained4 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondC604Lost
Hydrogen bondL610L610Preserved
Hydrogen bondR611R611Preserved
Polar contactV603Lost
Polar contactC604Lost
Polar contactL610L610Preserved
Polar contactR611R611Preserved
Van der WaalsR611Lost

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
0.03kcal/mol
Stabilising — mild
AlphaMissense
0.922
LPath
AlphaFold pLDDT
66
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditions(no specific conditions catalogued)
InheritanceNot specified.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00056%
cDNA changec.1820C>G
ClinVar accessionVCV001297726
Last evaluated2023/05/03 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 607 sits near the end of TM9. Neighbors: LEU608 (2.5 Å), VAL606 (2.5 Å), LEU610 (4.1 Å), SER605 (4.5 Å).

The wild-type proline likely defines a helix-end geometry. Replacing it with arginine removes the kink and introduces charge near the membrane-water interface. The near-zero ΔΔG reflects fold accommodation through the arginine extending toward solvent.

AlphaMissense 0.922 confirms severe consequence. P607L (Atlas card next) is the same position with leucine — both pathogenic.

Amino-acid chemistry
Proline (P) → Arginine (R) — rigid helix-breaking replaced by large positively-charged amine. Removes constraint, introduces charge into bilayer.
Position in the protein
TM9 (residues 589–609) · position 607 near the end of TM9 (pLDDT 66 borderline).

Druggability Assessment

Category 4 — Stable Fold, Function Disrupted. ΔΔG ≈ 0 — fold unchanged. AlphaMissense 0.922 confirms severe consequence.

Mechanism: loss of TM9 helix-end kink plus charge introduction. Therapeutic: site-directed at the TM9 C-terminal region.

Why this matters

P607R + P607L (next card) are both at position 607 — multi-substitution hotspot in TM9. First Atlas TM9 variants.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the P607R 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 P607R PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane589609 · Helical