Lysosomal storage disease

Lysosomal storage disease

Lysosomes are the primary disposal and recycling centres of cells.

It contains about 72 hydrolytic enzymes responsible for enzymatic degradation of various intracellular macromolecules (lipid, glycoprotein or mucopolysaccharides) and provides amino acids, fatty acids, nucleic acids and mono or disaccharides for reuse for cellular synthesis.

Lysosomal storage diseases are caused by lysosomal dysfunction usually due to deficiency of single enzyme leading to abnormal accumulation of catabolites of macromolecules.

About 3 dozens genetically determined, heterogenous, metabolic and acquired lysosomal disorders are classified as lysosomal storage diseases.

Lysosomal storage diseases are classified according to their major stored materials, e.g.,

•         Mucopolysaccharidosis (MPS),

•         Sphingolipidosis,

•         Galactosialidesis,

•         Sphingomyelin-cholesterol lipidosis,

•         Glycoprotein storage diseases,

•         Neuronal glycoproteinosis,

•         Glycogen storage diseases,

•         Neuronal ceroid-lipofuscinosis,

•         Drug induced lysosomal storage diseases.