Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system

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

Research output: Contribution to journalArticle

16 Citations (Scopus)

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 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
Pages (from-to)560-569
Number of pages10
JournalEuropean Journal of Neuroscience
Volume32
Issue number4
DOIs
StatePublished - Aug 2010

Fingerprint

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)
Mitochondrial Proteins
Nervous System
Dihydrolipoyllysine-Residue Acetyltransferase
Synaptosomal-Associated Protein 25
4-Nitrophenylphosphatase
Maple Syrup Urine Disease
Pyruvate Dehydrogenase Complex
Adrenergic Neurons
Branched Chain Amino Acids
Pyramidal Cells
Purkinje Cells
Dopaminergic Neurons
Brain
Motor Neurons
Mesencephalon
Cerebral Cortex
Cerebellum
Brain Stem
Spinal Cord

Keywords

  • Amino acid disorders
  • CNS
  • Mitochondria
  • Phenylketonuria

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Nautiyal, M., Sweatt, A. J., MacKenzie, J. A., Payne, R., Szucs, S., Matalon, R., ... Hutson, S. M. (2010). Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system. European Journal of Neuroscience, 32(4), 560-569. https://doi.org/10.1111/j.1460-9568.2010.07326.x

Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system. / Nautiyal, Manisha; Sweatt, Andrew J.; MacKenzie, James A.; Payne, R.; Szucs, Sylvia; Matalon, Reuben; Wallin, Reidar; Hutson, Susan M.

In: European Journal of Neuroscience, Vol. 32, No. 4, 08.2010, p. 560-569.

Research output: Contribution to journalArticle

Nautiyal, M, Sweatt, AJ, MacKenzie, JA, Payne, R, Szucs, S, Matalon, R, Wallin, R & Hutson, SM 2010, 'Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system', European Journal of Neuroscience, vol. 32, no. 4, pp. 560-569. https://doi.org/10.1111/j.1460-9568.2010.07326.x
Nautiyal, Manisha ; Sweatt, Andrew J. ; MacKenzie, James A. ; Payne, R. ; Szucs, Sylvia ; Matalon, Reuben ; Wallin, Reidar ; Hutson, Susan M. / Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system. In: European Journal of Neuroscience. 2010 ; Vol. 32, No. 4. pp. 560-569.
@article{01077a2e9e854d15a05d771f5a5625c3,
title = "Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system",
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 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.",
keywords = "Amino acid disorders, CNS, Mitochondria, Phenylketonuria",
author = "Manisha Nautiyal and Sweatt, {Andrew J.} and MacKenzie, {James A.} and R. Payne and Sylvia Szucs and Reuben Matalon and Reidar Wallin and Hutson, {Susan M.}",
year = "2010",
month = "8",
doi = "10.1111/j.1460-9568.2010.07326.x",
language = "English",
volume = "32",
pages = "560--569",
journal = "European Journal of Neuroscience",
issn = "0953-816X",
publisher = "Wiley-Blackwell",
number = "4",

}

TY - JOUR

T1 - Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system

AU - Nautiyal, Manisha

AU - Sweatt, Andrew J.

AU - MacKenzie, James A.

AU - Payne, R.

AU - Szucs, Sylvia

AU - Matalon, Reuben

AU - Wallin, Reidar

AU - Hutson, Susan M.

PY - 2010/8

Y1 - 2010/8

N2 - 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.

AB - 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.

KW - Amino acid disorders

KW - CNS

KW - Mitochondria

KW - Phenylketonuria

UR - http://www.scopus.com/inward/record.url?scp=77955748640&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77955748640&partnerID=8YFLogxK

U2 - 10.1111/j.1460-9568.2010.07326.x

DO - 10.1111/j.1460-9568.2010.07326.x

M3 - Article

VL - 32

SP - 560

EP - 569

JO - European Journal of Neuroscience

JF - European Journal of Neuroscience

SN - 0953-816X

IS - 4

ER -