The millennium King Arthur : the commodification of the Arthurian legend in the 20th century

The prophesy that King Arthur will return has come true. This legendary icon of Western civilization lives again in the popular culture novels of contemporary and futuristic literature. While the king’s personality has changed little since Malory, the monarch is now often found as a superhero in new world settings: he has become a Celtic space traveller among the stars, a modern politician fighting corruption, a WWII fighter pilot, a battler of aliens, and even returns as a teenage boy. Wherever he goes, King Arthur encounters a variety of personalised evil opponents from his medieval past as well as futuristic aliens and monsters. The authors and publishers of Arthurian popular culture have commodified the Arthurian legend, turning the king into an Americanised romantic superhero who overcomes his opponents but mostly fails to meet the reality of modern socio-economic challenges. The king has a limited understanding of what constitutes evil in the modern world so that despite his worthy character as a role model, his grasp of action required to overcome injustice constitutes a major shortcoming. The reasons for this are sought among the authors and publishers that produced these novels, and among the literary critics and the sociological literature focusing on the linkages between literature and society

The millennium King Arthur : the commodification of the Arthurian legend in the 20th century

The prophesy that King Arthur will return has come true. This legendary icon of Western civilization lives again in the popular culture novels of contemporary and futuristic literature. While the king’s personality has changed little since Malory, the monarch is now often found as a superhero in new world settings: he has become a Celtic space traveller among the stars, a modern politician fighting corruption, a WWII fighter pilot, a battler of aliens, and even returns as a teenage boy. Wherever he goes, King Arthur encounters a variety of personalised evil opponents from his medieval past as well as futuristic aliens and monsters. The authors and publishers of Arthurian popular culture have commodified the Arthurian legend, turning the king into an Americanised romantic superhero who overcomes his opponents but mostly fails to meet the reality of modern socio-economic challenges. The king has a limited understanding of what constitutes evil in the modern world so that despite his worthy character as a role model, his grasp of action required to overcome injustice constitutes a major shortcoming. The reasons for this are sought among the authors and publishers that produced these novels, and among the literary critics and the sociological literature focusing on the linkages between literature and society

Role of mitochondrial inner membrane organizing system in protein biogenesis of the mitochondrial outer membrane

Mitochondria contain two membranes, the outer membrane and the inner membrane with folded cristae. The mitochondrial inner membrane organizing system (MINOS) is a large protein complex required for maintaining inner membrane architecture. MINOS interacts with both preprotein transport machineries of the outer membrane, the translocase of the outer membrane (TOM) and the sorting and assembly machinery (SAM). It is unknown, however, whether MINOS plays a role in the biogenesis of outer membrane proteins. We have dissected the interaction of MINOS with TOM and SAM and report that MINOS binds to both translocases independently. MINOS binds to the SAM complex via the conserved polypeptide transport–associated domain of Sam50. Mitochondria lacking mitofilin, the large core subunit of MINOS, are impaired in the biogenesis of β-barrel proteins of the outer membrane, whereas mutant mitochondria lacking any of the other five MINOS subunits import β-barrel proteins in a manner similar to wild-type mitochondria. We show that mitofilin is required at an early stage of β-barrel biogenesis that includes the initial translocation through the TOM complex. We conclude that MINOS interacts with TOM and SAM independently and that the core subunit mitofilin is involved in biogenesis of outer membrane β-barrel proteins.

Mutations of the mitochondrial carrier translocase channel subunit TIM22 cause early-onset mitochondrial myopathy.

Protein import into mitochondria is facilitated by translocases within the outer and the inner mitochondrial membranes that are dedicated to a highly specific subset of client proteins. The mitochondrial carrier translocase (TIM22 complex) inserts multispanning proteins, such as mitochondrial metabolite carriers and translocase subunits (TIM23, TIM17A/B and TIM22), into the inner mitochondrial membrane. Both types of substrates are essential for mitochondrial metabolic function and biogenesis. Here, we report on a subject, diagnosed at 1.5 years, with a neuromuscular presentation, comprising hypotonia, gastroesophageal reflux disease and persistently elevated serum and Cerebrospinal fluid lactate (CSF). Patient fibroblasts displayed reduced oxidative capacity and altered mitochondrial morphology. Using trans-mitochondrial cybrid cell lines, we excluded a candidate variant in mitochondrial DNA as causative of these effects. Whole-exome sequencing identified compound heterozygous variants in the TIM22 gene (NM_013337), resulting in premature truncation in one allele (p.Tyr25Ter) and a point mutation in a conserved residue (p.Val33Leu), within the intermembrane space region, of the TIM22 protein in the second allele. Although mRNA transcripts of TIM22 were elevated, biochemical analyses revealed lower levels of TIM22 protein and an even greater deficiency of TIM22 complex formation. In agreement with a defect in carrier translocase function, carrier protein amounts in the inner membrane were found to be reduced. This is the first report of pathogenic variants in the TIM22 pore-forming subunit of the carrier translocase affecting the biogenesis of inner mitochondrial membrane proteins critical for metabolite exchange