Academic Journal
Molecular and cellular substrates for the Friedreich Ataxia. Significance of contactin expression and of antioxidant administration
| العنوان: | Molecular and cellular substrates for the Friedreich Ataxia. Significance of contactin expression and of antioxidant administration |
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| المؤلفون: | Bizzoca A., Caracciolo M., Corsi P., Magrone T., Jirillo E., Gennarini G. |
| المساهمون: | Bizzoca, A., Caracciolo, M., Corsi, P., Magrone, T., Jirillo, E., Gennarini, G. |
| سنة النشر: | 2020 |
| المجموعة: | Università degli Studi di Bari Aldo Moro: CINECA IRIS |
| مصطلحات موضوعية: | Friedreich Ataxia, Nervous tissue repair, Neural cells interaction, Neurodegeneration, Polyphenol, Transmembrane signaling |
| الوصف: | In this study, the neural phenotype is explored in rodent models of the spinocerebellar disorder known as the Friedreich Ataxia (FA), which results from mutations within the gene encoding the Frataxin mitochondrial protein. For this, the M12 line, bearing a targeted mutation, which disrupts the Frataxin gene exon 4 was used, together with the M02 line, which, in addition, is hemizygous for the human Frataxin gene mutation (Pook transgene), implying the occurrence of 82–190 GAA repeats within its first intron. The mutant mice phenotype was compared to the one of wild type littermates in regions undergoing differential profiles of neurogenesis, including the cerebellar cortex and the spinal cord by using neuronal (β-tubulin) and glial (Glial Fibrillary Acidic Protein) markers as well as the Contactin 1 axonal glycoprotein, involved in neurite growth control. Morphological/morphometric analyses revealed that while in Frataxin mutant mice the neuronal phenotype was significantly counteracted, a glial upregulation occurred at the same time. Furthermore, Contactin 1 downregulation suggested that changes in the underlying gene contributed to the disorder pathogenesis. Therefore, the FA phenotype implies an alteration of the developmental profile of neuronal and glial precursors. Finally, epigallocatechin gallate polyphenol administration counteracted the disorder, indicating protective effects of antioxidant administration. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| Relation: | info:eu-repo/semantics/altIdentifier/pmid/32906751; volume:25; issue:18; firstpage:4085; numberofpages:19; journal:MOLECULES; https://hdl.handle.net/11586/313259 |
| DOI: | 10.3390/molecules25184085 |
| الاتاحة: | https://hdl.handle.net/11586/313259 https://doi.org/10.3390/molecules25184085 |
| Rights: | info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.204CF93D |
| قاعدة البيانات: | BASE |
| DOI: | 10.3390/molecules25184085 |
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