Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …

Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and
involved in various aspects of cellular life, with a primary role in energy production. The …

The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …

Oligodendrocytes, the myelin-forming glial cells of the central nervous system, maintain long-
term axonal integrity,,. However, the underlying support mechanisms are not understood …

Mitochondrial dysfunction has gained recognition as a contributing factor in many diseases.
The kidney is a kind of organ with high energy demand, rich in mitochondria. As such …

2014 BMA Medical Book Awards Highly Commended in Pathology category! Muscle Biopsy:
A Practical Approach gives you all of the unparalleled guidance necessary to effectively …

Primary mitochondrial diseases are relatively common inborn errors of energy metabolism,
with a combined prevalence of 1 in 4300. These disorders typically affect tissues with high …

Leigh syndrome is a rare, complex, and incurable early onset (typically infant or early
childhood) mitochondrial disorder with both phenotypic and genetic heterogeneity. The …

The assembly of mitochondrial oxidative phosphorylation (OXPHOS) complexes is an
intricate process, which—given their dual-genetic control—requires tight co-regulation of two …

Mammalian mitochondrial DNA (mtDNA) is a small, maternally inherited genome that codes
for 13 essential proteins in the respiratory chain. Mature oocytes contain more than 150 000 …