Mitochondria is a key organelle to understand the pathogenesis of neurodegenerative disorders such as Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), and frontotemporal lobar degeneration (FTLD). Increased oxidative stress caused by mitochondrial degeneration has been reported in PD and ALS. The finding that mitochondrial toxins such as 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and rotenone reproduce the phenotypes in animal models of PD strengthens the involvement of mitochondria in neurodegeneration. Genetic factors responsible for these diseases further revealed that the dysfunction of mitochondrial respiratory activity, the dysregulation of redox status, the defects of mitochondrial quality control are closely linked to the disease etiology.
The early-onset Parkinson’s disease genes PINK1 and Parkin are involved in the mitochondrial quality control by suppression of the motility of damaged mitochondria in the neurons and removal of damaged mitochondria via mitophagy and in the translation regulation of certain nuclear-encoded mitochondrial genes. PD and ALS-FTLD-associated CHCHD2 and CHCHD10 regulate the mitochondrial respiratory activity and cristae organization. The accumulation of a-Synuclein, TDP-43, FUS and SOD1 in and out of mitochondria are pathological features of these diseases. However, due to multifaceted roles of mitochondria in neurons and glial cells, many unsolved questions remain in this research field.
This Research Topic is focused on the pathological role of mitochondria in neurodegenerative disorders including, but are not limited to, PD, ALS and FTDL. Original Research, Reviews, Methods and Opinion articles are welcome.
Mitochondria is a key organelle to understand the pathogenesis of neurodegenerative disorders such as Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), and frontotemporal lobar degeneration (FTLD). Increased oxidative stress caused by mitochondrial degeneration has been reported in PD and ALS. The finding that mitochondrial toxins such as 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and rotenone reproduce the phenotypes in animal models of PD strengthens the involvement of mitochondria in neurodegeneration. Genetic factors responsible for these diseases further revealed that the dysfunction of mitochondrial respiratory activity, the dysregulation of redox status, the defects of mitochondrial quality control are closely linked to the disease etiology.
The early-onset Parkinson’s disease genes PINK1 and Parkin are involved in the mitochondrial quality control by suppression of the motility of damaged mitochondria in the neurons and removal of damaged mitochondria via mitophagy and in the translation regulation of certain nuclear-encoded mitochondrial genes. PD and ALS-FTLD-associated CHCHD2 and CHCHD10 regulate the mitochondrial respiratory activity and cristae organization. The accumulation of a-Synuclein, TDP-43, FUS and SOD1 in and out of mitochondria are pathological features of these diseases. However, due to multifaceted roles of mitochondria in neurons and glial cells, many unsolved questions remain in this research field.
This Research Topic is focused on the pathological role of mitochondria in neurodegenerative disorders including, but are not limited to, PD, ALS and FTDL. Original Research, Reviews, Methods and Opinion articles are welcome.
