Myelin genes are downregulated in canine fucosidosis
| Autor: | Peter C. Thomson, Peter Williamson, Jessica L. Fletcher, Rosanne M. Taylor, Gauthami S. Kondagari, Amanda L. Wright |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
Fucosidosis
Down-Regulation Lysosomal storage disease Microarray Biology Canine disease model Myelin assembly Immunoenzyme Techniques Myelin Dogs Gene expression medicine Animals RNA Messenger Molecular Biology Neuroinflammation Oligonucleotide Array Sequence Analysis Inflammation Cell Death Reverse Transcriptase Polymerase Chain Reaction Gene Expression Profiling Neurodegeneration Brain medicine.disease Oligodendrocyte Cell biology Oligodendroglia medicine.anatomical_structure nervous system Immunology Molecular Medicine Hypomyelination Biomarkers Myelin Proteins |
| Zdroj: | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1812:1418-1426 |
| ISSN: | 0925-4439 |
| DOI: | 10.1016/j.bbadis.2011.06.001 |
| Popis: | The processes regulating the complex neurodegenerative cascade of vacuolation, neuroinflammation, neuronal loss and myelin deficits in fucosidosis, a neurological lysosomal storage disorder, remain unclear. To elucidate these processes the gene expression profile of the cerebral cortex from untreated and intrathecal enzyme replacement therapy treated fucosidosis pups and age-matched unaffected controls were examined. Neuroinflammation and cell death processes were identified to have a major role in fucosidosis pathophysiology with 37% of differentially expressed (DE) genes involved in these processes. Critical, specific, early decreases in expression levels of key genes in myelin assembly were identified by gene expression profiling, including myelin-associated glycoprotein (MAG), myelin and lymphocyte protein (MAL), and oligodendrocyte myelin paranodal and inner loop protein (OPALIN). These gene expression changes may be indicative of early neuronal loss causing reduced electrical impulses required for oligodendrocyte maturation. |
| Databáze: | OpenAIRE |
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