TSC1TSC2kompleksia

The TSC1TSC2 complex, also known as the tuberous sclerosis complex 1-2 complex, is a crucial intracellular regulator of cell growth and proliferation. This complex is formed by the proteins hamartin (encoded by TSC1) and tuberin (encoded by TSC2). These two proteins associate to form a heterodimer that functions as a tumor suppressor. The TSC1TSC2 complex acts by inhibiting the activity of the mechanistic target of rapamycin (mTOR) signaling pathway, a key pathway involved in cell growth, metabolism, and protein synthesis.

When the TSC1TSC2 complex is active, it phosphorylates and inactivates Rheb, a small GTPase that is a direct activator of mTORC1, a subset of mTOR. This inhibition of mTORC1 leads to reduced protein synthesis and cell growth. Conversely, mutations or loss of function in either TSC1 or TSC2 genes disrupt the formation or activity of the TSC1TSC2 complex. This loss of inhibition allows for uncontrolled activation of the mTOR pathway, promoting excessive cell growth and proliferation, which can lead to the development of tumors and hamartomas.

The TSC1TSC2 complex plays a vital role in preventing the uncontrolled cell division characteristic of cancers and developmental disorders. Dysregulation of this complex is directly implicated in tuberous sclerosis complex (TSC), a genetic disorder characterized by the growth of benign tumors in various organs, including the brain, skin, kidneys, and heart. Understanding the intricate functions of the TSC1TSC2 complex is fundamental to comprehending the pathogenesis of TSC and developing potential therapeutic strategies targeting the mTOR pathway.