National Film Archive

Calpainopathies, including limb-girdle muscular dystrophy recessive type 1 (LGMD R1) and the rare dominant type 4 (LGMD D4), are genetic neuromuscular disorders caused by pathogenic variants in the CAPN3 gene, which encodes calpain-3, a muscle-specific cysteine protease. This protein plays a crucial role in muscle remodelling, calcium homeostasis, and myogenesis regulation. LGMD R1, the most common form of LGMD, is characterized by progressive, symmetrical muscle weakness primarily affecting the shoulder and pelvic girdles, with an onset ranging from childhood to adulthood. It affects about 1 in 100,000 individuals. The clinical spectrum is wide, with three main phenotypes: pelvifemoral myopathy, scapulohumeral myopathy, and isolated hyperCKemia. LGMD D4 is characterized generally by a milder phenotype with variable but frequent axial muscle involvement. Diagnostic algorithm include serum CK levels dosage, muscle imaging, and sometimes a muscle biopsy, with definitive diagnosis confirmed by CAPN3 gene testing. Calpainopathies have no cure and care is focused on physiotherapy, management of muscle contractures, moderate physical activity, and orthosis. The newly established French National Diagnosis and Care Protocol (NDCP) aim to standardize the diagnosis and care of calpainopathies. The protocol emphasizes early diagnosis, personalized patient care, genetic assessment and prevention of disease progression. This initiative aims to reduce diagnostic delays and improve patient outcomes through a harmonized multidisciplinary approach, informed by the latest clinical and research expertise.

Resumo Nos estudos do cinema de horror mais recentes, evidencia-se a presença do subgênero slasher, conhecido pela presença de assassinatos em série e violência explícita em sua narrativa. Diante desse contexto, questiona-se: como as mulheres — e seus corpos — são representados em uma produção desse gênero? Para responder esse questionamento, utiliza-se como objeto deste estudo o filme X (2022), dirigido por Ti West. Este artigo objetiva compreender a representação da mulher no cinema de horror slasher, utilizando em seu corpo teórico os estudos relacionados ao horror e feminino. Espera-se, com este trabalho, colaborar com as discussões acadêmicas acerca dos estudos de gênero e sexualidade, feminismo e cinema de horror.

The burgeoning scholarship on Western health films stands in stark contrast to the vacuum in the historical conceptualization of Eastern European films. This book develops a nonlinear historical model that revises their unique role in the inception of national cinematography and establishing supranational health security. Readers witness the revelation of an unknown history concerning how the health films produced in Eastern European countries not only adopted Western patterns of propaganda but actively participated in its formation, especially with regard to those considered "others": Women and the populations of the periphery. The authors elaborate on the long "echo" of the discursive practices introduced by health films within public health propaganda, as well as the attempts to negate and deconstruct such practices by rebellious filmmakers. A wide range of methods, including the analysis of the sociological biographies of filmmakers, the historical reconstruction of public campaigns against diseases and an investigation into the production of health films, contextualizes these films along a multifaceted continuum stretching between the adaptation of global patterns and the cultivation of national authenticities. The book is aimed at those who study the history of film, the history of public health, Central and Eastern European countries and global history.

The aim of the article is to present the possibilities of the digitization of 3D objects using both professional and publicly accessible methods. The article also aims to focus on selected tools of 3D digitization and their possible use in the memory institutions, such as museum, libraries and archives.

Segundo de Chomón (1871-1929) est l’un des maîtres incontestés des premiers trucages cinématographiques et des débuts de la mise en couleurs des images animées. Néanmoins, ce pionnier espagnol est bien plus que cela et l’importance de son œuvre aurait sans doute été mieux étudiée sans l’ombre portée de Georges Méliès. Si Chomón a pu s’inspirer du célèbre prestidigitateur français dans certains de ses films à trucs, il s’en distingue toutefois clairement par son exploitation magistrale du tour de manivelle, des ombres chinoises et du mouvement inversé. Par ailleurs, il reste l’un des rares à avoir réussi le passage entre le cinéma monstratif des films à trucs des années 1900 et le cinéma institutionnalisé des années 1910. Les trucages de ses premières scènes à trucs chez Pathé frères deviendront effets spéciaux dans les films narratifs dont il assurera l’exécution, tel Maciste alpino de Giovanni Pastrone en 1916. Cet ouvrage propose de revisiter son œuvre et de comprendre les mille et un visages de ce formidable pionnier du cinématographe, truqueur, coloriste et cinématographiste. Cet ouvrage est issu d’un colloque organisé en novembre 2017 par la Fondation Jérôme Seydoux-Pathé et « Les Arts trompeurs. Machines. Magie. Médias ».

Am Beispiel des wenig bekannten Films Stekljannyi glaz / Das Glasauge von Lilja Brik (1891-1978) aus dem Jahre 1929 befasst sich dieser Aufsatz mit dem Aufkommen weiblicher Autorschaft in den Kreisen der sowjetischen Avantgarde. Lilja Briks Werke wurden durch den Formalismus/ Konstruktivismus motiviert und sind als eine Kritik der burgerlichen Ideologie zu verstehen. Ihre kunstlerische Herangehensweise kann in fortlaufender Entwicklung als postmodern “avant la lettre” betrachtet werden, in der das jeweilige Thema in einem faszinierenden Denkprozess von Zitat und Parodie entwickelt wird. Ihre vermeintliche Vorliebe fur kreative Montage und Rekombination fuhrt von einer weiblich kodierten Unterordnung weg und wird dabei zum tatsachlichen Ausdruck ihres individuellen Stils.

Analysis of music in "Ur-Hamlet"

Within the anime evolution, Kenzo Masaoka name is one of the most forgotten. The great pioneer and explorer of cell and sound techniques, Masaoka made a risky and transcendental decision when he decided to abandon the most traditional stream of Japanese animation, which resulted in an enormous budget increase. Well versed in both Eastern and Western Painting, his relevance in the configuration of the anime and also his contribution to the assimilation and development of foreign techniques and the introduction of new narrative motives indicate him as the key figure of Toei Animation’s birth.