Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system.
作者： Manisha Nautiyal , Andrew J. Sweatt , James A. MacKenzie , R. Mark Payne , Sylvia Szucs
DOI: 10.1111/J.1460-9568.2010.07326.X
关键词:
摘要: The NIPSNAP (4-nitrophenylphosphatase domain and non-neuronal SNAP25-like protein homolog 1) proteins belong to a highly conserved family of unknown function. We found that NIPSNAP1 binds the branched-chain α-keto acid (BCKA) dehydrogenase enzyme complex, which is disrupted in maple syrup urine disease, disease amino catabolism results neurological dysfunction. Phenylketonuric (PKU) epileptic mice show altered expression brain. Therefore, distribution localization rat brain was determined. Results expressed exclusively neurons including pyramidal cerebral cortex, Purkinje cerebellum motor spinal cord. Dopaminergic midbrain noradrenergic brainstem, are affected PKU, also express NIPSNAP1. be localized mitochondrial matrix can bind dihydrolipoyl-transacylase -transacetylase components BCKA pyruvate complexes vitro. Our data provide first experimental evidence for strictly neuronal this nervous system.
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