Abstract Mitochondria contain about 1,000–1,500 proteins that fulfil multiple functions.
Mitochondrial proteins originate from two genomes: mitochondrial and nuclear. Hence …

Mitochondrial diseases are a group of genetic disorders that are characterized by defects in
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …

Hereditary spastic paraplegia (HSP) describes a heterogeneous group of genetic
neurodegenerative diseases characterised by progressive spasticity of the lower limbs. The …

Objective The prevalence of mitochondrial disease has proven difficult to establish,
predominantly as a result of clinical and genetic heterogeneity. The phenotypic spectrum of …

Mitochondria form a highly interconnected tubular network throughout the cell via a dynamic
process, with mitochondrial segments fusing and breaking apart continuously. Strong …

Recent advances in mitochondrial biology have revealed the high diversity and complexity
of proteolytic enzymes that regulate mitochondrial function. We have classified mitochondrial …

Mitochondria are essential metabolic hubs that dynamically adapt to physiological demands.
More than 40 proteases residing in different compartments of mitochondria, termed …

Mitochondrial diseases are among the most common and most complex of all inherited
genetic diseases. The involvement of both the mitochondrial and nuclear genome presents …

Mitochondrial disorders have emerged as a common cause of inherited disease, but are
traditionally viewed as being difficult to diagnose clinically, and even more difficult to …

Hereditary spastic paraplegia comprises a wide and heterogeneous group of inherited
neurodegenerative and neurodevelopmental disorders resulting from primary retrograde …