Overview

The brain is divided into gray matter where neurons are located and white matter that connects the neurons.

White matter diseases are disorders in which the parts of the white matter do not form normally or become degenerated or damaged over time.

There are various types of this diseases, including congenital types that develop during the early stages of development and acquired types that develop in adulthood.

Characteristics

The characteristics of white matter diseases are assumed to include abnormalities related to the formation and maintenance of myelin sheaths, which are the main structural component of white matter, neurotrophic disorders that result in inadequate oxygen and nutrient supply to neurons, destruction of myelin sheaths due to inflammation, and decreased blood flow to white matter.

Congenital hereditary white matter diseases are thought to be caused by damage to glial cells (astrocytes, oligodendrocytes, and microglia) present in white matter.

Research status

Hereditary white matter diseases include Alexander's disease, Pelizaeus-Merzbacher's disease, and lysosomal storage disease. Although genetic mutations associated with these diseases are known, the pathogenic mechanisms are still not fully understood.

Using iPS cells derived from patients with white matter disease, the molecular mechanisms of white matter disease are being elucidated, and applied to the development of treatments.

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