Alteration of the glutamate and GABA transporters in the hippocampus of the Niemann-Pick disease, type C mouse using proteomic analysis

Kyunghee Byun, Jaewoo Kim, Sang Yun Cho, Brian Hutchinson, Se Ran Yang, Kyung Sun Kang, Moonjae Cho, Kyukye Hwang, Makoto Michikawa, Young Wook Jeon, Young Ki Paik, Bonghee Lee

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23 Citations (Scopus)

Abstract

Niemann-Pick disease type C (NPC) is a fatal autosomal recessive cholesterol disorder characterized by severe progressive neurodegeneration. To unveil the mechanism of neurodegeneration, proteomic and morphological approaches were applied to the hippocampus in NPC -/- mouse. Two-DE was utilized to resolve the hippocampal protein expression profiles of 4- and 8-week-old NPC +/+ and -/- mice. Differentially expressed protein spots were identified by MALDI-TOF MS and database searching. At 4 weeks of age, there was no significant difference in protein profiles between NPC +/+ and -/- mice. However, at the age of 8 weeks, NPC +/+ and -/- mice showed marked difference in protein expressions. Among these, glutamate receptor 2 precursor was identified. The immunohistochemical study on neurotransporters showed that glial GABA transporter (GAT-3) increased in both 4- and 8-week-old NPC -/-mouse and glutamic acid decarboxylase (GAD-6) increased in 8-week-old NPC -/- mouse. Glial glutamate transporter, excitatory amino acids carrier-1 (EAAC1), decreased in 8-week-old NPC -/- mouse. In conclusion, our data may provide insight into the understanding of the basic mechanism through perturbation of protein networks and neurotransporter systems in a single gene knockout model of NPC disease.

Original languageEnglish
Pages (from-to)1230-1236
Number of pages7
JournalProteomics
Volume6
Issue number4
DOIs
Publication statusPublished - 2006 Feb 1

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Biology

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    Byun, K., Kim, J., Cho, S. Y., Hutchinson, B., Yang, S. R., Kang, K. S., Cho, M., Hwang, K., Michikawa, M., Jeon, Y. W., Paik, Y. K., & Lee, B. (2006). Alteration of the glutamate and GABA transporters in the hippocampus of the Niemann-Pick disease, type C mouse using proteomic analysis. Proteomics, 6(4), 1230-1236. https://doi.org/10.1002/pmic.200500412