TRABD modulates mitochondrial homeostasis and tissue integrity

Cell Rep. 2024 Jun 25;43(6):114304. doi: 10.1016/j.celrep.2024.114304. Epub 2024 Jun 5.

Abstract

High TRABD expression is associated with tau pathology in patients with Alzheimer's disease; however, the function of TRABD is unknown. Human TRABD encodes a mitochondrial outer-membrane protein. The loss of TRABD resulted in mitochondrial fragmentation, and TRABD overexpression led to mitochondrial clustering and fusion. The C-terminal tail of the TRABD anchored to the mitochondrial outer membrane and the TraB domain could form homocomplexes. Additionally, TRABD forms complexes with MFN2, MIGA2, and PLD6 to facilitate mitochondrial fusion. Flies lacking dTRABD are viable and have normal lifespans. However, aging flies exhibit reduced climbing ability and abnormal mitochondrial morphology in their muscles. The expression of dTRABD is increased in aged flies. dTRABD overexpression leads to neurodegeneration and enhances tau toxicity in fly eyes. The overexpression of dTRABD also increased reactive oxygen species (ROS), ATP production, and protein turnover in the mitochondria. This study suggested that TRABD-induced mitochondrial malfunctions contribute to age-related neurodegeneration.

Keywords: CP: Cell biology; Drosophila; mitochondria; mitochondria fusion; neurodegeneration.

MeSH terms

  • Aging / metabolism
  • Animals
  • Drosophila Proteins / genetics
  • Drosophila Proteins / metabolism
  • Drosophila melanogaster* / metabolism
  • GTP Phosphohydrolases / metabolism
  • Homeostasis*
  • Humans
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism
  • Mitochondria* / metabolism
  • Mitochondrial Dynamics
  • Mitochondrial Membranes / metabolism
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism
  • Reactive Oxygen Species* / metabolism
  • tau Proteins / metabolism

Substances

  • Reactive Oxygen Species
  • tau Proteins
  • Drosophila Proteins
  • Mitochondrial Proteins
  • Membrane Proteins
  • GTP Phosphohydrolases