PEX26-F51L Research

Computational and structural analysis of a peroxisomal disease variant

Overview

Peroxisome biogenesis factor 26 (PEX26) is a tail-anchored membrane protein essential for peroxisome biogenesis. It serves as the membrane anchor for the AAA-ATPase complex PEX1/PEX6, which drives recycling of the peroxisomal targeting receptor PEX5.

The missense variant F51L in PEX26 is associated with peroxisome biogenesis disorders on the Zellweger spectrum, and has been linked to sensorineural hearing loss.

This site presents ongoing computational structural analysis of this variant, including AlphaFold 3 modeling, CHARMM-GUI system preparation, GROMACS molecular dynamics simulations, and BioEmu conformational ensemble generation.

Key Questions

• How does F51L alter the structure and dynamics of the PEX26 cytosolic domain?
• Does the mutation affect recruitment or binding of the PEX1/PEX6 complex?
• How is this related to sensorineural hearing loss?

Status

Work in progress — structural models and ensemble analysis ongoing.