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showed that, in mice, thiamine deprivation for 14 days led to different degrees of enzymatic deficiencies when testing for TKT, PDH, and KGDH activities in the cortex and hippocampus ( In cases of severe thiamine deficiency, however, a broad enzymatic deficiency encompassing all three mitochondrial TPP-dependent enzymes would be expected to result in symptoms similar to those of the genetic disorder dihydrolipoamide dehydrogenase (DLDD) deficiency (OMIM 246900)

[DOI] [PMC free article] [PubMed] [Google Scholar] 31.El Chemali L., Boutary S., Liu S., Liu G.J., Middleton R.J., Banati R.B., Bahrenberg G., Rupprecht R., Schumacher M., Massaad-Massade L