In silico Study of the Uncertainly Significant VCP Variants reveal major Structural, Functional fluctuations leading to potential Disease-Based Associations
Abstract
Valosin containing protein is involved in a plethora of crucial functions from proteostasis, stress granule clearance to genome maintenance and ubiquitination. Hence, mutations in VCP can lead to a plethora of fatal diseases like Amyotrophic Lateral Sclerosis, Inclusion body myopathy with Paget disease of bone and frontotemporal dementia type 1, Spastic paraplegia, Charcot-Marie-Tooth disease type 2Y, Dementia, and Osteitis Deformans to name a few. Studies on VCPs disease phenotype relationship, structural, and functional modifications of the proteins stability, conservation, molecular dynamics, and post-translational modifications havent been performed. This in silico study investigated the variants of VCP (R95C, R95G, A160P, R191P, R191Q) which have conflicting interpretations of pathogenicity which are often depreciated and lack data. Additionally, this study screens the study cohort and the fatal diseases linked to all these variants. Interestingly through various computational tools and disease-based population studies, it was found that these variants are often found in patients linked with fatal diseases. The protein-protein interaction showed UFD1 has a direct association with VCP. The physicochemical parameters showed that A160P had the highest fluctuations of all the variants. VCPs protein secondary structure, molecular dynamics simulations of RMSD, RMSF, RoG, hydrogen bonds, and solvent accessibility were all comparatively impacted due to the changes caused by the variants. Box-plot and Principal component analysis of the MD simulations visualized the changes in the wild type of the protein. Research in wet labs and screening of patient cohorts is necessary to further characterize the diseases linked with these variants. This can potentially lead to the identification of biomarkers for fatal rare diseases.
02 /Specification & Classification
Click any tag to browse matching papers- Citations
- 1
- Organism
- Assay
- Research Focus
- Approach
Reproducibility ledger
Nobody has reported trying to reproduce this paper yet. Did you replicate an experiment or run the code? Your report saves the next lab a week.
Sign in to file a reproducibility report.
Related papers
- A Novel Autosomal Dominant Childhood-Onset Disorder Associated with Pathogenic Variants in VCP
Annelise Yoo Mah-Som, Jil Daw, … Marwan S. Shinawi·medRxiv·Jun 19, 2023
- Genetic analysis of VCP variants in a Turkish dementia cohort
Nadia Dehghani, Zeynep Tüfekçıoğlu, … José Brás·medRxiv·Feb 13, 2023
- Utilization of CoRDS Registry to Monitor Quality of Life in Patients with VCP Multisystem Proteinopathy
Eiman Abdoalsadig, Merwa Hamid, … Virginia E. Kimonis·Orphanet Journal of Rare DiseasesPeer-reviewed·Apr 14, 2025
- ASPL-driven subunit exchange remodels VCP/p97 hexamers and is impaired by a multisystem proteinopathy mutation
Jingxuan Tang, Laxmikanta Khamari, … Nils G. Walter·bioRxiv·May 8, 2026
- Fatty links between multisystem proteinopathy and Small VCP-Interacting Protein
F. Ramzan, F. Abrar, … D. D. O. Martin·bioRxiv·Mar 31, 2023
- Complementary roles of structure and variant effect predictors in RyR1 clinical interpretation
Rolando Hernández Trapero, Mihaly Badonyi, … Joseph A. Marsh·Human MutationPeer-reviewed·Jan 1, 2025