Altered Expression of DJ-1 and PINK1 in Sporadic ALS and in the SOD1G93AALS Mouse Model
| Autor: | Julia Sipos, Nadine Thau-Habermann, Reinhard Dengler, Sarah Knippenberg, Sonja Körner, Klaus Jan Rath, Susanne Petri |
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| Rok vydání: | 2013 |
| Předmět: |
Adult
Male Genetically modified mouse Muscle tissue Pathology medicine.medical_specialty Protein Deglycase DJ-1 SOD1 Mice Transgenic PINK1 Biology Pathology and Forensic Medicine Pathogenesis Mice Cellular and Molecular Neuroscience Gastrocnemius muscle Superoxide Dismutase-1 medicine Animals Humans Amyotrophic lateral sclerosis Muscle Skeletal Aged Motor Neurons Oncogene Proteins Muscle biopsy medicine.diagnostic_test Superoxide Dismutase Amyotrophic Lateral Sclerosis Intracellular Signaling Peptides and Proteins Brain General Medicine Middle Aged medicine.disease Mitochondria nervous system diseases Disease Models Animal medicine.anatomical_structure Spinal Cord Neurology Female Neurology (clinical) Protein Kinases |
| Zdroj: | Journal of Neuropathology & Experimental Neurology. 72:1052-1061 |
| ISSN: | 1554-6578 0022-3069 |
| DOI: | 10.1097/nen.0000000000000004 |
| Popis: | Mitochondrial dysfunction is an important mechanism in the pathogenesis of neurodegenerative diseases such as Parkinson disease and amyotrophic lateral sclerosis (ALS). DJ-1 and PTEN-induced putative kinase 1 (PINK1) are important proteins for the maintenance of mitochondrial function and protection against cell death. Mutations in the genes coding for these proteins cause familial forms of Parkinson disease. Recent studies have postulated that changes in the expression of both proteins are also involved in pathologic mechanisms in ALS mouse models. Here, we studied the mRNA and protein expression of PINK1 and DJ-1 in postmortem brain and spinal cord tissue and muscle biopsy samples from ALS patients and controls and in brain, spinal cord, and gastrocnemius muscle of SOD1(G93A) ALS mice at different disease stages. We found significant decreases of PINK1 and DJ-1 mRNA levels in muscle tissue of SOD1(G93A) mice. Together with the significant decrease of PINK1 mRNA levels in human ALS muscle tissue, statistically nonsignificant reduction of DJ-1 mRNA levels, and reduced immunostaining for PINK1 in human ALS muscle, the results suggest potential pathophysiologic roles for these proteins in both mutant SOD1 transgenic mice and in sporadic ALS(G93A). |
| Databáze: | OpenAIRE |
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