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Fig. 1 | Fluids and Barriers of the CNS

Fig. 1

From: The neurovascular unit in leukodystrophies: towards solving the puzzle

Fig. 1

Schematic representation of models to study the NVU in leukodystrophies in humans. The first step of studying leukodystrophies is in a clinical setting, using MRI and next-generation sequencing for initial diagnosis, monitoring disease progression and treatment of patients. Since most leukodystrophies are fatal, post-mortem analysis and post-mortem tissue-derived primary cells, immortalized cell lines, and organotypic slice cultures are valuable tools to distinguish primary cellular processes involved in the pathogenesis. Finally, hiPSC-based models can contribute to investigating the molecular pathways and dynamics at the NVU during disease development and test therapeutic interventions. Together, clinical and basic research can contribute to understanding disease mechanisms in leukodystrophies

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