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P607L

Category 4 — Stable Fold, Function DisruptedConflictingTransmembrane · predictedEditorial
ProlineLeucine at position 607 · TM9 (589-609), helical transmembrane · WFS1 (Wolframin)

Proline → Leucine at position 607 inside TM9. ClinVar Conflicting including monogenic diabetes, T2D, DFNA6. AlphaMissense 0.416 (below threshold), ΔΔG -0.27. Same position as P607R — proline-removal pair.

Interactive 3D Structure

Wild-type reference
Wild-type P607 — hydrogen bond to R611
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DynaMut2 mutant · P607L
Mutant L607 — hydrogen bond to C604 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost1 gained4 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondC604Lost
Hydrogen bondL610L610Preserved
Hydrogen bondR611R611Preserved
Polar contactV603Lost
Polar contactC604Lost
Polar contactL610L610Preserved
Polar contactR611R611Preserved
Van der WaalsR611Lost
HydrophobicL610Gained

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
-0.27kcal/mol
Destabilising — mild
AlphaMissense
0.416
Amb
AlphaFold pLDDT
66
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsMonogenic diabetes; Type 2 diabetes mellitus; Autosomal dominant nonsyndromic hearing loss 6 (DFNA6)
InheritanceMulti-phenotype AD.
Population frequency (gnomAD v4)Ultra-rare · AF 0.0021%
cDNA changec.1820C>T
ClinVar accessionVCV000045441
Last evaluated2025/12/29 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 607 same neighbors as P607R: LEU608 (2.5 Å), VAL606 (2.5 Å), LEU610 (4.1 Å), SER605 (4.5 Å).

P607L is the second pathogenic substitution at 607 (with P607R). Both remove the wild-type proline kink at TM9's end. P607L is the conservative hydrophobic substitution; P607R adds charge.

AM 0.416 below threshold but multi-phenotype confirms pathogenicity.

Amino-acid chemistry
Proline (P) → Leucine (L) — rigid helix-breaking replaced by branched aliphatic.
Position in the protein
TM9 (residues 589–609) · position 607 (pLDDT 66 borderline). Same as P607R.

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| 0.27. AlphaMissense 0.416 below threshold but multi-phenotype confirms pathogenicity.

Mechanism: same TM9 kink-removal as P607R. Therapeutic: TM9 site-directed.

Why this matters

P607L + P607R confirm position 607 as TM9 multi-substitution hotspot.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the P607L 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 P607L PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane589609 · Helical