Blood-brain barrier defects associated with Rbp9 mutation

Jihyun Kim, Young Joon Kim, Jeongsil Kim-Ha

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Rbp9 is a Drosophila RNA-binding protein that shares a high level of sequence similarity with Drosophila elav and human Hu proteins. Loss of function alleles of elav are embryonic lethal causing abnormal central nervous system (CNS) development, and Hu is implicated in the development of paraneoplastic neurological syndrome associated with small cell lung cancer. To elucidate the role of Rbp9, we generated Rbp9 mutant flies and examined them for symptoms related to paraneoplastic encephalomyelitis. Although Rbp9 proteins begin to appear from the middle of the pupal period in the cortex of the CNS, the Rbp9 mutants showed no apparent defects in development. However, as the mutant adult flies grew older, they showed reduced locomotor activities and lived only one-half of the life expectancy of wild-type flies. To understand the molecular mechanism underlying this symptom, gene expression profiles in Rbp9 mutants were analyzed and potential target genes were further characterized. Reduced expression of cell adhesion molecules was detected, and defects in the blood-brain barrier (BBB) of Rbp9 mutant brains could be seen. Putative Rbp9-binding sites were found in introns of genes that function in cell adhesion. Therefore, Rbp9 may regulate the splicing of cell adhesion molecules, critical for the formation of the BBB.

Original languageEnglish
Pages (from-to)93-98
Number of pages6
JournalMolecules and cells
Volume29
Issue number1
DOIs
Publication statusPublished - 2010 Jan 1

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Blood-Brain Barrier
Diptera
Cell Adhesion Molecules
Mutation
Nervous System Paraneoplastic Syndromes
Central Nervous System
Drosophila Proteins
Paraneoplastic Syndromes
RNA-Binding Proteins
Small Cell Lung Carcinoma
Locomotion
Life Expectancy
Transcriptome
Cell Adhesion
Introns
Genes
Drosophila
Half-Life
Proteins
Alleles

All Science Journal Classification (ASJC) codes

  • Molecular Biology
  • Cell Biology

Cite this

Kim, Jihyun ; Kim, Young Joon ; Kim-Ha, Jeongsil. / Blood-brain barrier defects associated with Rbp9 mutation. In: Molecules and cells. 2010 ; Vol. 29, No. 1. pp. 93-98.
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Blood-brain barrier defects associated with Rbp9 mutation. / Kim, Jihyun; Kim, Young Joon; Kim-Ha, Jeongsil.

In: Molecules and cells, Vol. 29, No. 1, 01.01.2010, p. 93-98.

Research output: Contribution to journalArticle

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