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F882C

Category 4 — Stable Fold, Function DisruptedLikely pathogenicLumenal · predictedσ-1 candidateEditorial
PhenylalanineCysteine at position 882 · TM11 (870-890), helical transmembrane · WFS1 (Wolframin)

Phenylalanine → Cysteine at position 882 inside TM11. ClinVar Likely pathogenic, auditory neuropathy. AlphaMissense 0.496 (below threshold) — AM under-call. DynaMut2 ΔΔG -1.59 kcal/mol (destabilising). Volume loss in the TM11 aromatic cluster.

Interactive 3D Structure

Wild-type reference
Wild-type F882 — hydrogen bond to A878
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DynaMut2 mutant · F882C
Mutant C882 — hydrogen bond to A878 lost (4 contacts lost)
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Bond changes · DynaMut2 interaction analysis

4 lost2 gained6 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondY508Gained
Hydrogen bondA878A878Preserved
Hydrogen bondF879F879Preserved
Polar contactY508Y508Preserved
Polar contactA878A878Preserved
Polar contactF879F879Preserved
Polar contactD880D880Preserved
Van der WaalsI416Lost
Van der WaalsA878Lost
Van der WaalsD880Gained
HydrophobicI416Lost
HydrophobicM539Lost

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
-1.59kcal/mol
Destabilising — moderate
AlphaMissense
0.496
Amb
AlphaFold pLDDT
82
model confidence
Schema
Cat 4
Category 4 — Stable Fold, Function Disrupted

Clinical Evidence

ClinVar classificationLikely pathogenic
Review statuscriteria provided, single submitter
Associated conditionsAuditory neuropathy
InheritanceAuditory neuropathy documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00012%
cDNA changec.2645T>G
ClinVar accessionVCV002683881
Last evaluated2023/12/22 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 882 sits inside TM11. The AlphaFold model places F882 within 5 Å of PHE881 (2.5 Å), PHE883 (2.5 Å), PHE879 (3.9 Å), ASP880 (4.2 Å), and ALA878 (4.4 Å). The local environment is a dense aromatic cluster — four phenylalanines (F879, F881, F882, F883) plus the nearby P885 (P885L Atlas card) and F884/F886 (in the broader cluster).

Replacing F882 with cysteine eliminates one of the four aromatics in this cluster. The π-stacking network reorganizes; the introduced thiol can engage in oxidative disulfide chemistry if a partner cysteine is nearby (none within 5 Å here). The |ΔΔG| of 1.59 reflects substantial structural cost from the lost aromatic.

AlphaMissense's 0.496 is below the 0.564 likely-pathogenic threshold — another AM under-call case. ClinVar Likely Pathogenic + auditory neuropathy + the substantial ΔΔG argue for genuine pathogenicity despite the AM signal.

Amino-acid chemistry
Phenylalanine (F) → Cysteine (C) — aromatic hydrophobic replaced by thiol-bearing residue. Massive volume loss; aromatic character lost.
Position in the protein
TM11 (residues 870–890) · position 882 mid-helix, bilayer-embedded (pLDDT 82).

Druggability Assessment

Category 3/4 — Most Druggable (AM under-call). |ΔΔG| = 1.59 — close to Cat 2. AlphaMissense 0.496 below threshold but ClinVar pathogenic + auditory neuropathy + substantial ΔΔG confirm pathogenicity.

Mechanism is loss of one aromatic from the dense TM11 phenylalanine cluster (F879-F881-F882-F883). Therapeutic strategy: site-directed at the TM11 aromatic cluster — same broader region as P885L.

Why this matters

F882C joins the AM-under-call class (with W639G, R629W, E202G). Drug discovery should integrate ΔΔG with AM rather than treating either as the sole pathogenicity signal.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the F882C 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 F882C PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Transmembrane870890 · Helical