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Bioenergetic dysfunction in a zebrafish model of acute hyperammonemic decompensation

Matthias Zielonka1, Joris Probst2, Matthias Carl3

  • 1Center for Child and Adolescent Medicine, Division for Pediatric Neurology and Metabolic Medicine, University Hospital Heidelberg, Heidelberg, Germany; Heidelberg Research Center for Molecular Medicine (HRCMM), Heidelberg, Germany.

Experimental Neurology
|January 18, 2019
PubMed
Summary

Acute hyperammonemia impairs brain energy production by depleting 2-oxoglutarate from the TCA cycle. This leads to ATP shortage and neurotoxicity in urea cycle disorders.

Keywords:
Bioenergetic impairmentHyperammonemiaNeurotoxicityUrea cycle disordersZebrafish

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