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 and 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 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. APP metabolism and β-amyloid accumulation are suspected to influence mitochondrial functions. The accumulation of α-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.
Besides the well-known role as powerhouses of eukaryotic cells, mitochondria can also function as metabolism and signal transduction hubs. The communications between mitochondria, other organelles and cellular proteostasis that are essential for maintaining the integrity of structure and functions of neurons, are also weighted heavily in the pathogenesis of human neurodegenerative diseases.
Following the success of the first volume Molecular Links between Mitochondrial Damage and Parkinson's Disease and Related Disorders, this research topic is aimed at providing researchers with an exclusive platform to publish their latest discoveries in the pathological role of mitochondria in neurodegenerative disorders including, but are not limited to, AD, PD, ALS and FTLD, in addition to discussing the concerns and advances in the field through reviews, brief research reports and opinion articles. Topics of interest for volume II may include, but are not limited to:
• Molecular mechanisms of neurodegeneration caused by mitochondrial degeneration
• The correlation between mitochondrial function and protein homeostasis (proteostasis)
• The interaction between mitochondria and other organelles (such as ER, Golgi apparatus, peroxisome, lysosome)
• The mitochondrial communication with the nucleus, such as retrograde signaling pathways
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 and 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 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. APP metabolism and β-amyloid accumulation are suspected to influence mitochondrial functions. The accumulation of α-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.
Besides the well-known role as powerhouses of eukaryotic cells, mitochondria can also function as metabolism and signal transduction hubs. The communications between mitochondria, other organelles and cellular proteostasis that are essential for maintaining the integrity of structure and functions of neurons, are also weighted heavily in the pathogenesis of human neurodegenerative diseases.
Following the success of the first volume Molecular Links between Mitochondrial Damage and Parkinson's Disease and Related Disorders, this research topic is aimed at providing researchers with an exclusive platform to publish their latest discoveries in the pathological role of mitochondria in neurodegenerative disorders including, but are not limited to, AD, PD, ALS and FTLD, in addition to discussing the concerns and advances in the field through reviews, brief research reports and opinion articles. Topics of interest for volume II may include, but are not limited to:
• Molecular mechanisms of neurodegeneration caused by mitochondrial degeneration
• The correlation between mitochondrial function and protein homeostasis (proteostasis)
• The interaction between mitochondria and other organelles (such as ER, Golgi apparatus, peroxisome, lysosome)
• The mitochondrial communication with the nucleus, such as retrograde signaling pathways
