BBSome: a New Player in Hypertension and Other Cardiovascular Risks

The BBSome is an octameric protein complex involved in Bardet-Biedl syndrome (BBS), a human pleiotropic, autosomal recessive condition. Patients with BBS display various clinical features including obesity, hypertension, and renal abnormalities. Association studies have also linked the BBS genes to hypertension and other cardiovascular risks in the general population. The BBSome was originally associated with the function of cilia, a highly specialized organelle that extend from the cell membrane of most vertebrate cells. However, subsequent studies have implicated the BBSome in the control of a myriad of other cellular processes not related to cilia including cell membrane localization of receptors and gene expression. The development of animal models of BBS such as mouse lines lacking various components of the BBSome and associated proteins has facilitated studying their role in the control of cardiovascular function and deciphering the pathophysiological mechanisms responsible for the cardiovascular aberrations associated with BBS. These studies revealed the importance of the neuronal, renal, vascular, and cardiac BBSome in the regulation of blood pressure, renal function, vascular reactivity, and cardiac development. The BBSome has also emerged as a critical regulator of key systems involved in cardiovascular control including the renin-angiotensin system. Better understanding of the influence of the BBSome on the molecular and physiological processes relevant to cardiovascular health and disease has the potential of identifying novel mechanisms underlying hypertension and other cardiovascular risks.

Automated summary

The BBSome is a protein complex associated with Bardet-Biedl syndrome (BBS) and has been linked to various cardiovascular conditions. It was initially believed to be involved in cilia function, but further studies have shown its role in multiple cellular processes, including receptor localization and gene expression. Animal models have helped uncover the importance of the BBSome in cardiovascular regulation, such as blood pressure control, renal function, vascular reactivity, and cardiac development. The BBSome also plays a critical role in key systems involved in cardiovascular control, including the renin-angiotensin system.