Lysosomes are somewhat large organelles, formed by the rugged endoplasmic reticulum stored in the Golgi complex. They are spherical surrounded by membrane capable of performing metabolism or intracellular digestion of macromolecules in a controlled manner.
Their dysfunction causes diseases, some of which are very well known, but their main manifestations start in adulthood, such as Gaucher’s disease or Fabry’s disease, they are liposomal diseases .
Other diseases, such as mucopolysaccharidosis, some cerolipofuscinosis or ASMD, Acid sphingomyelinase deficiency (ASMD) or Niemann-Pick disease, are diagnosed very early and effective treatment can be initiated.
Hydrolytic and proteolytic enzymes that serve to digest materials of external origin (heterophagy) or internal origin (autophagy) found in the body.
They have a high content of acid hydrolases (more than 40). Lysosomal enzymes can digest bacteria and other substances that enter the cell by phagocytosis, or other processes of endocytosis.
Mannose-6-P is the molecular marker, the seal, that directs enzymes to the lysosome route.
Lysosomal enzymes are recognized and selectively marked by adding mannose 6-phosphate (M6P) to the N-oligosaccharide added in the endoplasmic reticulum, during its passage to the Golgi apparatus.
The sperm acrosome is a special lysosome containing hyaluronidase, protease and abundant acid phosphatase.
The most important enzymes of the lysosome are
Lipases, which digest lipids: esterases, phospholipases
Glucosidases, which digest carbohydrates: glycogen
Proteases, which digest proteins: peptides
Nucleases, which digest nucleic acids, RNA and DNA
Phosphatases: Acid phosphatase
Lysosomes essentially act as the cell’s digestive system and hydrogen ions maintain the acidity of the inner fluid.
When foreign pathogens, such as bacteria, enter a cell, lysosomes can help neutralize them by digesting them. In this way they help with an organism’s immune response.
If lysosomes do not function properly, they can cause lysosomal storage disorders or diseases.
If one of the many lysosomal enzymes has an incorrect chemical formula due to a genetic mutation, the substance that should metabolize or destroy the enzyme accumulates.
Progranulin (PGRN) is a cytokine, formed in the adipose tissue, that participates in the biogenesis of lysosomes.
-Kazmi F et al., (2013) Drug Metab Dispos 41; 897-905
-Sardiello M, Palmieri M, di Ronza A, Medina DL, Valenza M, Gennarino VA, Di Malta C, Donaudy F, Embrione V, Polishchuk RS, Banfi S, Parenti G, Cattaneo E, Ballabio A (July 2009). «A gene network regulating lysosomal biogenesis and function». Science. 325
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