Lysosomal storage disorders and iron.

摘要: Lysosomal storage disorders are a group of about 50 rare metabolic diseases that result from defects in lysosomal function. The majority is recessively inherited and caused by mutations genes encoding proteins as the basis for its pathobiology. lysosome plays pivotal role cell's ability to recycle degrade unwanted material. One functions relates regulating iron levels throughout body. Iron double-edged sword: It absolutely required an organism's survival, but high quickly lead cell death. In addition, recent results have put on map pathways leading common neurodegenerative diseases, such Parkinson's disease Alzheimer's disease. plausible mechanisms through which acts these also involve this would significant implications our understanding molecular etiology disorders.