Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system

Manisha Nautiyal, Andrew J. Sweatt, James A. MacKenzie, R. Mark Payne, Sylvia Szucs, Reuben Matalon, Reidar Wallin, Susan M. Hutson

Research output: Contribution to journalArticlepeer-review

18 Scopus citations


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 α-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.

Original languageEnglish (US)
Pages (from-to)560-569
Number of pages10
JournalEuropean Journal of Neuroscience
Issue number4
StatePublished - Aug 2010


  • Amino acid disorders
  • CNS
  • Mitochondria
  • Phenylketonuria

ASJC Scopus subject areas

  • General Neuroscience


Dive into the research topics of 'Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system'. Together they form a unique fingerprint.

Cite this