TSC1

TSC1 functions as a co-chaperone which inhibits the ATPase activity of the chaperone Hsp90 (heat shock protein-90) and decelerates its chaperone cycle. TSC1 functions as a facilitator of Hsp90 in chaperoning the kinase and non-kinase clients including TSC2, therefore preventing their ubiquitination and degradation in the proteasome.^[6] TSC1, TSC2 and TBC1D7 is a multi-protein complex also known as the TSC complex. This complex negatively regulates mTORC1 signaling by functioning as a GTPase-activating protein (GAP) for the small GTPase Rheb, an essential activator of mTORC1. The TSC complex has been implicated as a tumor suppressor.

Defects in this gene can cause tuberous sclerosis, due to a functional impairment of the TSC complex.^{[citation needed]} Defects in TSC1 may also be a cause of focal cortical dysplasia.^{[citation needed]} TSC1 may be involved in protecting brain neurons in the CA3 region of the hippocampus from the effects of stroke.^[7]

TSC1 has been shown to interact with:

• AKT1,^[8]
• HSP70^[6]
• HSP90^[6]
• NEFL,^[9]
• PLK1,^[10] and
• TSC2.^{[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][excessive citations]}

• Tuberous sclerosis protein