Abstract Background Mutation of the m. 8993 T > G ATP6 subunit ATP synthase causes a maternally inherited Leigh Syndrome (LS), rapidly fatal encephalomyelopathy in childhood called and related Neuropathy, Ataxia Retinitis Pigmentosa (NARP) adults. There is no cure, relevant animal models are few. Here we describe novel mouse model LS preclinical efficacy gene therapy approach to neutralize m...

BACKGROUND AND PURPOSE Subacute necrotizing encephalomyelopathy, or Leigh syndrome (LS), is a progressive neurodegenerative disorder characterized by symmetrical spongiform lesions in the brain with onset usually in infancy or early childhood. Little is known of the developing process of the brain lesions in LS that are particularly relevant to the occurrence of fatal respiratory failure. Our p...

The chimeric murine oncornavirus FrCas(E) causes a rapidly progressive noninflammatory spongiform encephalomyelopathy after neonatal inoculation. The virus was constructed by the introduction of pol-env sequences from the wild mouse virus CasBrE into the genome of a neuroinvasive but nonneurovirulent strain of Friend murine leukemia virus (FMuLV), FB29. Although the brain infection by FrCas(E) ...

The alphaherpesvirus, equine herpesvirus type 1 (EHV-1), is a highly prevalent cause of equine infectious abortion and encephalomyelopathy. These syndromes have been attributed to ischemic necrosis from thrombosis in placental and neural vessels, although the mechanisms underlying thrombosis are unknown. After inhalation, EHV-1 establishes a peripheral blood mononuclear cell-associated viremia,...

Mosquito-borne flaviviruses are a major current and emerging threat, affecting millions of people worldwide. Global climate change, combined with increasing proximity of humans to animals and mosquito vectors by expansion into natural habitats, coupled with the increase in international travel, have resulted in significant spread and concomitant increase in the incidence of infection and severe...

UNLABELLED Equine herpesvirus type 1 (EHV-1) is a main cause of respiratory disease, abortion, and encephalomyelopathy in horses. Monocytic cells (CD172a(+)) are the main carrier cells of EHV-1 during primary infection and are proposed to serve as a "Trojan horse" to facilitate the dissemination of EHV-1 to target organs. However, the mechanism by which EHV-1 is transferred from CD172a(+) cells...

The clinical and pathological features of multiple neurofibromatosis (von Recklinghausen's disease) are usually striking and often rather bizarre. It has hence attracted much interest and is well described and illustrated. Recent accounts include those by Willis (1958) and Russell and Rubinstein (1959); a fuller review in German by Schmincke (1956) is mainly devoted to the description and inter...