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A study by the Consejo Superior de Investigaciones Científicas (CSIC) provides new data on the complement system, a basic component of the immune system of the human being, he acts out uncontrolled, can cause diseases such as macular degeneration the second cause of blindness in older people.

The work, published in the latest issue of the journal Proceedings of the National Academy of Sciences USA, focuses on the AP C3 convertase, a complex enzyme composed of two proteins key to the complement system responds quickly and effectively, eliminating pathogenic micro-organism or substance which led to its activation. The results help to determine its potential as a therapeutic target in diseases caused by failures of the complement system.

Teams Santiago Rodríguez de Córdoba and Oscar Llorca at the Centro de Investigaciones Biológicas (CSIC) in Madrid, how they have managed to display assembles and regulates the AP C3 convertase when the complement system is activated by one of three ways possible, the alternative route. Rodriguez de Cordoba explains the motivations of the study: "The complement system is a double edged sword and the AP C3 convertase their most dangerous tool. Alterations in this complex cause deregulation of the supplement and therefore damage to tissues or organs" .

Therefore, the researcher added CSIC, training and regulation of the AP C3 convertase is "exquisite", so that the plug can fulfill its mission, eliminating a quick and efficient foreign substances to the body, while the acting on the tissues are protected from destruction by complement.

In addition to the degeneration of the macula (the area of the retina specialized in fine detail vision, which you can read or see the face of a person, for example), the deregulation of the complement system via the AP C3 convertase leads to some rare renal diseases, such as atypical hemolytic uremic syndrome, or dense deposit disease. "The AP C3 convertase is potentially an important therapeutic target, and they know how it is regulated and how is critical," concludes Rodriguez de Cordoba.

The formation of the AP C3 convertase is initiated with the association of two proteins that give rise to complex C3bB inactive. Then, through the action of a novel protein, and after several moves, the convertase is activated. The research carried out by groups and Rodríguez de Córdoba Llorca has made a detailed way how it is produced and how C3bB activation of the inactive complex to generate active AP C3 convertase.

For this, the authors have used electron microscopy have shown that with the thousands of complex C3bB. These individual images were used later to reconstruct the complex three-dimensional inactive and active AP C3 convertase.

"Knowing how it assembles and how it regulates the AP C3 convertase will help greatly to determine its potential as a therapeutic target, facilitating, for example, the design and development of compounds that inhibit their formation or accelerate its dissociation and thus prevent or reduce tissue damage caused by the uncontrolled activation of the complement system, "explains researcher CSIC.