2 ms·
Understanding Causes of Devastating Neurodegenerative Condition on Children
- gardenfelder 3y ago>Lack of CLN2 protein. Golgi bodies create lysosomal enzymes. Green proteins are in the membrane of the Golgi. Lysosomal enzyme transport from the Golgi to the lysosomes is disrupted leading to defective lysosomes and disdirectred enzymes. Researchers studied the lack of functional CLN3 protein, which underlies Batten disease. They found lack of the protein leads to a breakdown of the M6PR receptor (green) in the lysosomes and subsequent disruption of needed lysosomal enzymes and the formation of normal lysosomes.