2. Academic Validation
  3. The Reversible Carnitine Palmitoyltransferase 1 Inhibitor (Teglicar) Ameliorates the Neurodegenerative Phenotype in a Drosophila Huntington's Disease Model by Acting on the Expression of Carnitine-Related Genes

The Reversible Carnitine Palmitoyltransferase 1 Inhibitor (Teglicar) Ameliorates the Neurodegenerative Phenotype in a Drosophila Huntington's Disease Model by Acting on the Expression of Carnitine-Related Genes

  • Molecules. 2022 May 13;27(10):3125. doi: 10.3390/molecules27103125.
Carla Bertapelle 1 2,  Maria Rosaria Carillo 1 3,  Nunzio Antonio Cacciola 1 4,  Yulii V Shidlovskii 5 6,  Gianfranco Peluso 1,  Filomena Anna Digilio 1
Affiliations

Affiliations

  • 1 Research Institute on Terrestrial Ecosystems (IRET), UOS Naples-CNR, Via Pietro Castellino 111, 80131 Naples, Italy.
  • 2 Department of Neurosciences, Reproductive and Odontostomatological Sciences, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy.
  • 3 Department of Experimental Medicine, University of Campania "Luigi Vanvitelli", Via Santa Maria di Costantinopoli 16, 80138 Naples, Italy.
  • 4 Department of Veterinary Medicine and Animal Productions, University of Naples Federico II, Via Pansini 5, 80131 Naples, Italy.
  • 5 Department of Gene Expression Regulation in Development, Institute of Gene Biology, Russian Academy of Sciences, 34/5 Vavilov St., 119334 Moscow, Russia.
  • 6 Department of Biology and General Genetics, I.M. Sechenov First Moscow State Medical University, 8, bldg. 2 Trubetskaya St., 119048 Moscow, Russia.
PMID: 35630602 DOI: 10.3390/molecules27103125
Abstract

Huntington's disease (HD) is a dramatic neurodegenerative disorder caused by the abnormal expansion of a CAG triplet in the Huntingtin gene, producing an abnormal protein. As it leads to the death of neurons in the cerebral cortex, the patients primarily present with neurological symptoms, but recently metabolic changes resulting from mitochondrial dysfunction have been identified as novel pathological features. The carnitine shuttle is a complex consisting of three Enzymes whose function is to transport the long-chain fatty acids into the mitochondria. Here, its pharmacological modification was used to test the hypothesis that shifting metabolism to lipid oxidation exacerbates the HD symptoms. Behavioural and transcriptional analyses were carried out on HD Drosophila model, to evaluate the involvement of the carnitine cycle in this pathogenesis. Pharmacological inhibition of CPT1, the rate-limiting Enzyme of the carnitine cycle, ameliorates the HD symptoms in Drosophila, likely acting on the expression of carnitine-related genes.

Keywords

Drosophila melanogaster; Huntington’s disease; L-carnitine; carnitine shuttle; mitochondria; neurodegeneration; β-oxidation.

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