Tissue-specific differences in the assembly of mitochondrial Complex I are revealed by a novel ENU mutation in ECSIT.

ABSTRACT:

Aims

Mitochondrial Complex I assembly (MCIA) is a multi-step process that necessitates the involvement of a variety of assembly factors and chaperones to ensure that the final active enzyme is correctly assembled. The role of the assembly factor evolutionarily conserved signalling intermediate in the toll (ECSIT) pathway was studied across various murine tissues to determine its role in this process and how this varied between tissues of varying energetic demands. We hypothesized that many of the known functions of ECSIT were unhindered by the introduction of an ENU-induced mutation, while its role in Complex I assembly was affected on a tissue-specific basis.

Methods and results

Here, we describe a mutation in the MCIA factor ECSIT that reveals tissue-specific requirements

SUBMITTER: Nicol T 

PROVIDER: S-EPMC10578914 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature