Cellular and molecular mechanisms of aspartoacylase and its role in Canavan disease

Abstract Canavan disease is an autosomal recessive and lethal neurological disorder, characterized by the spongy degeneration of the white matter in the brain. The disease is caused by a deficiency of the cytosolic aspartoacylase (ASPA) enzyme, which catalyzes the hydrolysis of N-acetyl-aspartate (N...

Full description

Bibliographic Details
Main Authors: Martin Grønbæk-Thygesen, Rasmus Hartmann-Petersen
Format: Article
Language:English
Published: BMC 2024-04-01
Series:Cell & Bioscience
Subjects:
Online Access:https://doi.org/10.1186/s13578-024-01224-6