Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system

The European Journal of Neuroscience
Manisha NautiyalSusan M Hutson

Abstract

The NIPSNAP (4-nitrophenylphosphatase domain and non-neuronal SNAP25-like protein homolog 1) proteins belong to a highly conserved family of proteins of unknown function. We found that NIPSNAP1 binds to the branched-chain alpha-keto acid (BCKA) dehydrogenase enzyme complex, which is disrupted in maple syrup urine disease, a disease of branched-chain amino acid catabolism that results in neurological dysfunction. Phenylketonuric (PKU) and epileptic mice show altered expression of NIPSNAP1 in the brain. Therefore, the distribution and localization of NIPSNAP1 in rat brain was determined. Results show that NIPSNAP1 is expressed exclusively in neurons including pyramidal neurons in the cerebral cortex, Purkinje neurons in the cerebellum and motor neurons in the spinal cord. Dopaminergic neurons in midbrain and noradrenergic neurons in the brainstem, which are affected in PKU, also express NIPSNAP1. NIPSNAP1 is found to be localized in the mitochondrial matrix and can bind dihydrolipoyl-transacylase and -transacetylase components of the BCKA and pyruvate dehydrogenase complexes in vitro. Our data provide the first experimental evidence for a strictly neuronal expression of this mitochondrial protein in the rat nervous system.

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Citations

Feb 9, 2012·The Journal of Biological Chemistry·Emiko Okuda-AshitakaSeiji Ito
Aug 24, 2012·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·Manisha NautiyalDebra I Diz
Feb 19, 2014·Molecular and Cellular Neurosciences·Jens WeingartenWalter Volknandt
May 26, 2012·Channels·Joel M BrittainRajesh Khanna
Apr 1, 2016·Molecular Pain·Kazuya OkamotoEmiko Okuda-Ashitaka
Apr 24, 2014·Proteomes·Melanie LaßekWalter Volknandt
Jul 12, 2019·Journal of Molecular Biology·Chantell S Evans, Erika L F Holzbaur
Apr 15, 2021·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Esmat FathiRamin Homayouni

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