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Bestrophin-1 鈥� The Protein

The BEST1 gene gives instruction to make the BEST1 protein.

Proteins are complex molecules that do the work in cells, playing a role in almost every biological process. Proteins are required for cell structure and function and they help regulate processes in our tissues and organs. The exact structure and function of听the BEST1听protein听has been a cause for much debate in previous years, with most scientists suggesting it may have multiple purposes within the RPE.

BEST1 belongs to the Bestrophin protein family and is made up of 585 amino acids听(the building blocks of proteins) and extensive research has shown that it is located exclusively in the Retinal Pigment Epithelium (RPE) of the eye. Studies have shown that BEST1 protein听is located听at听the听basolateral听membrane听of the RPE听I.e.听along the base and up the sides听of听the听RPE.听听

BEST1听is believed to听act as a听channel听protein,听controlling听the flow of听chloride ions (small atoms with an electric charge)听through the basolateral membrane听of the RPE cell.听The听BEST1听channel acts as a gate,听which is听only opened when a calcium ion binds to听it. BEST1 is therefore known as a calcium-activated chloride channel.听

Chloride is an essential electrolyte that helps to maintain fluid balance in the body, it also plays a role in the generation of electrical signals in cells.听The听controlled听movement听of chloride ions听by BEST1 could听act to听regulate the听transport of fluid in and out of听cells听or听generate听an听electrical message.听

Schematic showing BEST1 protein located on the basal side of the RPE. Open channels are shown by a bound calcium ion, these allow the flow of chloride ions into the RPE. Closed BEST1 channels are shown by the absence of a bound calcium ion.
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Research has shown that within RPE听cells,听the听BEST1听protein may be involved in a number of processes, including听the regulation and recruitment of听calcium ions, the听movement of other molecules within听and into听the RPE,听and听maintaining the normal听shape听of cells听in certain conditions.听BEST1听may also听play a role in the normal development of the retina.听

Away from the eye, research has shown that the protein may also act as a channel for molecules bigger than chloride ions and even be involved in neurotransmitter (chemical messengers used to convey signals in the central nervous system) release. This has led researchers to study how BEST1 works in the brain.听Animal studies,听involving mice,听have also shown potential roles for BEST1 in the body outside of the eye听and research is on-going to understand the complete picture of BEST1 function.听

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