Mouse developmental defects, but not paraganglioma tumorigenesis, upon conditional Complex II loss in early Sox10⁺ cells.

ABSTRACT: In humans, loss of heterozygosity for defective alleles of any of the four subunits of mitochondrial tricarboxylic acid cycle enzyme succinate dehydrogenase (SDH, also Complex II of the electron transport chain) can lead to paraganglioma tumors in neuroendocrine cells. With the goal of developing mouse models of this rare disorder, we have developed various SDH conditional loss strategies. Based on recent lineage tracing studies, we hypothesized that conditional SDHC loss in early embryogenesis during migration of primordial neural crest cells that form the susceptible chromaffin cells of the adrenal medulla might induce paraganglioma. We triggered low levels of detectable SDHC loss in Sox10⁺ cells at E11.5 of mouse development. We report that, rather than developing adrenal med

SUBMITTER: Lewis EP 

PROVIDER: S-EPMC11467736 | biostudies-literature | 2024 Sep

REPOSITORIES: biostudies-literature