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VCP R155H

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R155H IBMPFD / ALS / FTD P55072 September 03, 2026
Average Confidence: 83.0%

01/3D Structure

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? About the 3D Viewer

Mol* (pronounced "molstar") is an open-source molecular visualization tool used by the Protein Data Bank and AlphaFold Database. Learn more at molstar.org.

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What am I looking at?

This is a predicted 3D structure of the protein. The ribbon diagram shows the protein backbone—helices appear as coils, sheets as arrows, and loops as simple lines. The shape determines how the protein functions: where it binds to other molecules, how it catalyzes reactions, and how mutations might disrupt its activity.

Color legend:

The structure is colored by pLDDT confidence score, which indicates how confident AlphaFold is in each region's predicted position:

  • Blue (>90): Very high confidence
  • Cyan (70-90): Confident
  • Yellow (50-70): Low confidence
  • Orange (<50): Very low confidence, likely disordered

02/AI Analysis

AI summary not yet generated. Check back soon.

03/Research Data

ClinVar Classification

Not found in ClinVar

ClinVar Database

Population Frequency

6.84e-07

Extremely rare (<0.01%)

AC: 1 / AN: 1461880

gnomAD Browser

Disease Associations

2081 total
inclusion body myopathy with Paget disease of bone and frontotemporal dementia type 1
0.80
literature: 0.05 animal model: 0.44 genetic association: 0.95 genetic literature: 0.73
frontotemporal dementia and/or amyotrophic lateral sclerosis 6
0.77
animal model: 0.42 genetic association: 0.90 genetic literature: 0.78
inclusion body myopathy with Paget disease of bone and frontotemporal dementia
0.74
literature: 0.55 animal model: 0.63 genetic association: 0.85 genetic literature: 0.76
Charcot-Marie-Tooth disease type 2Y
0.70
literature: 0.04 animal model: 0.48 genetic association: 0.79 genetic literature: 0.67
amyotrophic lateral sclerosis
0.67
literature: 0.95 animal model: 0.54 genetic association: 0.65 genetic literature: 0.85

Showing 5 of 2081 associations

Open Targets Platform

AI Research Brief

Research brief will be generated when agent findings are available.

04/AlphaFold Metrics

No visualization images available.

05/Domain Annotations

Structural Domains & Regions

residues 708–727 Region — Disordered
residues 768–806 Region — Disordered
residues 797–806 Region — Interaction with UBXN6
residues 802–806 Motif — PIM motif
residues 777–793 Compositional bias — Gly residues

Functional Sites

residues 247–253 Binding site
residue 348 Binding site
residue 384 Binding site
residues 521–526 Binding site

Binding Partners

ASPSCR1 (36 experiments)
NSFL1C (28 experiments)
UBXN6 (26 experiments)
UBXN2A (20 experiments)
UBXN7 (19 experiments)
NPLOC4 (17 experiments)
ATXN3 (16 experiments)
FAF2 (16 experiments)
UBXN2B (16 experiments)
AMFR (12 experiments)

Gene Ontology

ATPase complex GO:1904949 azurophil granule lumen GO:0035578 cytoplasm GO:0005737 cytoplasmic stress granule GO:0010494 cytoplasmic ubiquitin ligase complex GO:0000153 cytosol GO:0005829 Derlin-1 retrotranslocation complex GO:0036513 endoplasmic reticulum GO:0005783 endoplasmic reticulum membrane GO:0005789 extracellular exosome GO:0070062 extracellular region GO:0005576 ficolin-1-rich granule lumen GO:1904813 glutamatergic synapse GO:0098978 intracellular membrane-bounded organelle GO:0043231 lipid droplet GO:0005811 +76 more

06/Structural Caption

Structured caption not yet generated. Check back after the next fold analysis.

07/Peptide Therapeutics

Aggregation Analysis

Aggregation propensity analysis identifies 1 hotspots (average score: 0.02) using Pawar+KyteDoolittle+charge algorithm.

Residues 265–269 (0.80)

08/Known Inhibitors

Known Binders from ChEMBL

CHEMBL2311578 IC50: 24.0 nM (pChEMBL 7.62)

CHEMBL2311578

CHEMBL2315422 IC50: 25.0 nM (pChEMBL 7.6)

CHEMBL2315422

CHEMBL2315430 IC50: 41.0 nM (pChEMBL 7.39)

CHEMBL2315430

CHEMBL2315431 IC50: 42.0 nM (pChEMBL 7.38)

CHEMBL2315431

CHEMBL2315424 IC50: 53.0 nM (pChEMBL 7.28)

CHEMBL2315424

CHEMBL2315423 IC50: 54.0 nM (pChEMBL 7.27)

CHEMBL2315423

CHEMBL2315421 IC50: 58.0 nM (pChEMBL 7.24)

CHEMBL2315421

CHEMBL2315432 IC50: 63.0 nM (pChEMBL 7.2)

CHEMBL2315432

CHEMBL2315433 IC50: 65.0 nM (pChEMBL 7.19)

CHEMBL2315433

CHEMBL2315425 IC50: 67.0 nM (pChEMBL 7.17)

CHEMBL2315425

09/Candidate Peptides

De Novo Peptide Design Pipeline

Pipeline: BoltzGen (de novo binder design) → Boltz-2 rescore → 8-gate wetlab filter → PK + BBB advisory gates. Target site selected from UniProt curated annotations, P2Rank pocket prediction, and aggregation propensity (in that priority order). Advisory gates annotate each candidate with estimated serum half-life, renal/immunogenicity risk, and (for CNS targets) a recommended blood-brain-barrier shuttle conjugation — without silently dropping designs.

Loading candidate statistics...

Sequences are withheld pending IP review. Full candidate data (sequences, scores, CIF files) is available to authorized reviewers via the /api/private/candidates/{fold_id} endpoint with X-Private-Key.

Legacy candidates (charge-complementary)

Target Region

Residues 265–269 (0.80 aggregation score)

Candidate ID

CP-VCP-001 (7 residues · computational design)
âš  Drug-likeness concerns Stability: medium | Toxicity: low
t½ ≈ 2 min renal high ⚙ mods suggested 🧠 Glutathione conjugate 👃 intranasal option

10/Agent Findings

3 findings Last updated:
Clinical: 1 Supplements: 1 Peptides: 1

Clinical Agent (1)

Clinical Agent

First baseline data collection

Supplements Agent (1)

Supplements Agent

No analysis available.

Peptide Agent (1)

Peptide Agent

VCP R155H: 10 known binders (top: 24.0 nM); 1 candidate peptides designed