Botulinum neurotoxins (BoNTs) and tetanus toxin (TeTX) are the deadliest biological substances that cause botulism and tetanus, respectively. Their astonishing potency and capacity to enter neurons and interfere with neurotransmitter release at presynaptic terminals have attracted much interest in experimental neurobiology and clinical research. Fused with reporter proteins or labelled with fluorophores, BoNTs and TeTX and their non-toxic fragments also offer remarkable opportunities to visualize cellular processes and functions in neurons and synaptic connections. This study presents the state-of-the-art optical probes derived from BoNTs and TeTX and discusses their applications in molecular and synaptic biology and neurodevelopmental research. It reviews the principles of the design and production of probes, revisits their applications with advantages and limitations and considers prospects for future improvements. The versatile characteristics of discussed probes and reporters make them an integral part of the expanding toolkit for molecular neuroimaging, promoting the discovery process in neurobiology and translational neurosciences.

• Klíčová slova
• Advanced biomaterials, Fluorescent probes, Fusion proteins, Molecular trafficking, Optical imaging, Retrograde transport, SNARE proteins,
• MeSH
• botulotoxiny chemie   MeSH
• fluorescenční barviva chemie   MeSH
• lidé MeSH
• molekulární sondy chemie   MeSH
• neurony * metabolismus   MeSH
• neurotoxiny * MeSH
• neurozobrazování * metody   MeSH
• synapse * metabolismus   MeSH
• tetanový toxin * chemie   MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• botulotoxiny MeSH
• fluorescenční barviva MeSH
• molekulární sondy MeSH
• neurotoxiny * MeSH
• tetanový toxin * MeSH

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative condition characterised by a progressive loss of motor neurons controlling voluntary muscle activity. The disease manifests through a variety of motor dysfunctions related to the extent of damage and loss of neurons at different anatomical locations. Despite extensive research, it remains unclear why some motor neurons are especially susceptible to the disease, while others are affected less or even spared. In this article, we review the neurobiological mechanisms, neurochemical profiles, and morpho-functional characteristics of various motor neuron groups and types of motor units implicated in their differential exposure to degeneration. We discuss specific cell-autonomous (intrinsic) and extrinsic factors influencing the vulnerability gradient of motor units and motor neuron types to ALS, with their impact on disease manifestation, course, and prognosis, as revealed in preclinical and clinical studies. We consider the outstanding challenges and emerging opportunities for interpreting the phenotypic and mechanistic variability of the disease to identify targets for clinical interventions.

• Klíčová slova
• Bulbar and spinal ALS, Excitotoxicity, Motor neuron disease, Motor units, SOD1 mutation, Skeletal and visceral muscles, TDP43,
• MeSH
• amyotrofická laterální skleróza * MeSH
• lidé MeSH
• modely nemocí na zvířatech MeSH
• motorické neurony MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH