Article
Mitochondrial succinate is instrumental for HIF1alpha nuclear translocation in SDHA-mutant fibroblasts under normoxic conditions.
Human molecular genetics - 1 Nov 2005
Brière Jean-Jacques, Favier Judith, Bénit Paule, El Ghouzzi Vincent, Lorenzato Annalisa, Rabier Daniel, Di Renzo Maria Flavia, Gimenez-Roqueplo Anne-Paule, Rustin Pierre
Abstract excerpt
The genes encoding succinate dehydrogenase (SDH) subunits B, C and D, act as tumour suppressors in neuro-endocrine tissues. Tumour formation has been associated with succinate accumulation. In paraganglioma cells, two forms of SDHA (type I, II) were found which might preclude significant succinate accumulation in the case of a mutation in either form. In fibroblasts only SDHA type I is found. In these cells, SDHA...
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