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F882C

Category 4 — Stable Fold, Function DisruptedLikely pathogenicTransmembrane · predictedEditorial
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
ΔΔG confidence: Reduced confidence · Transmembrane
  • transmembrane position; predictor benchmarked on soluble proteins

This variant sits in the transmembrane band. The stability value was computed with a predictor benchmarked on water-soluble proteins. Published benchmarks of ddG predictors on membrane proteins report correlation below 0.4. Treat the magnitude as unreliable and the sign as weak evidence only.

Do not rank this value against a variant from a different region on ΔΔG alone.

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.

Classified for1★ unverified submission

ClinVar classifies this variant as Likely pathogenic for Hearing loss, inheritance unstated (inheritance not specified).

  • Hearing loss, inheritance unstatedinheritance not specifiedsubmitted as: Auditory neuropathy
  • This rests on a single submitter (1★). Treat the conditions above as submitted, not as documented phenotypes.
  • Phenotype scope is interpretation of submitted records. The computed values on this card (AlphaMissense, DynaMut2 ΔΔG, pLDDT) are measurements of the model, not of a patient.
  • Presentation cannot be generalised from neighbouring variants: one nucleotide over can present very differently. Structural similarity supports a mechanistic hypothesis, never a phenotype claim.

Review status: 1★ single submitter. ClinVar "conditions" is the union of all submissions; 0–1★ entries are submitted conditions, not documented phenotypes.

Population frequency
Global (all ancestries)
AF 0.00012% · 2 / 1,613,074 alleles
Homozygotes
0
Highest-frequency population
East Asian · AF 0.0045%

Highest in East Asian: AF 0.0045% (2 of 44,890 alleles), 35.9x the global figure. The global AF describes the general population, not the at-risk group.

No homozygotes reported in gnomAD v4. For a recessive allele this is expected and is not, on its own, evidence either way.

Ancestry groupAllele freq.AC / ANHom.Carrier est.
East Asian0.0045%2 / 44,8900~1 in 11220

Source: gnomAD r4, retrieved 2026-08-21. Values are allele frequencies. The carrier estimate is derived from the allele frequency (Hardy-Weinberg, ~2·AF) and is an approximation, not a published figure — where a published carrier frequency exists it outranks this column and is cited on the card.

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