Topic: mitochondrial fusion

Charcot Marie Tooth (CMT) disease is a rare and incurable genetic disease of the Peripheral Nervous System resulting from defects in Schwann cells (demyelinating CMT) or in motor/sensory neurons (axonal CMT). More than 120 causative CMT-related genes have been identified so far. Among them, CMT type 2A is the most common axonal form and is linked to mutations in the MFN2 gene encoding Mitofusin-2 (MFN2). MFN2 is an important regulator of mitochondrial fusion. Around a hundred mutations have been described and are distributed throughout all the protein's domains. Consequently, gene therapy stra

Christianson syndrome (CS) is an X-linked monogenic neurodevelopmental/neurodegenerative disorder arising from loss of function mutations in the endosomal pH regulator, Na+, K+/H+ exchanger isoform 6 (NHE6) and is estimated to afflict 1 in 16,000–100,000 people worldwide. In addition to significant limitations in cognitive and adaptive abilities, all affected males have ataxia. Mitochondrial dysfunction is increasingly being recognized as an important factor contributing to the pathogenesis of ataxia. In particular, dysregulated mitochondrial fusion and fission events can now be regarded as pl

Neurogenesis is the process by which dividing neural stem/progenitor cells (NSPCs) produce post-mitotic cells. The balance between self-renewal and differentiation is a highly controlled process under the influence of intrinsic and extrinsic factors. Previous studies have pointed to a model whereby cell fate is largely determined before the last mitosis. However, recent findings indicate that in the cerebral cortex mitochondrial dynamics have an impact on neurogenesis after the last cell division. Daughter cells with high levels of mitochondrial fusion undergo self-renewal, whereas those cells

Some studies demonstrate the beneficial role of high doses of vitamin B12 (VitB12) supplementation for the recovery from specific neurological impairments; however, the detailed mechanisms are not clearly elucidated. With this in mind, we employed an in vitro cell model made of hydrogen peroxide-insulted differentiated-SH-SY5Y (exhibiting a neuron-like phenotype) to study the effect of both a high and the physiological dose of VitB12 during recovery. The physiological dose of VitB12 showed an improvement in the cell count (although cell viability, evaluated by MTT, was comparably decreased in