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COPII
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'''COPIIββ''' is a type of [[coat protein]] that is involved in the transport of proteins from the [[endoplasmic reticulum]] (ER) to the [[Golgi apparatus]]. This process is known as [[ER to Golgi transport]], which is a crucial step in the [[secretory pathway]] of eukaryotic cells. == Structure == COPII is composed of five different proteins: [[Sec23]], [[Sec24]], [[Sec13]], [[Sec31]], and [[Sar1]]. These proteins assemble into a coat that surrounds transport vesicles as they bud from the ER. The assembly of the COPII coat is regulated by the [[GTPase]] activity of Sar1. == Function == The primary function of COPII is to transport proteins from the ER to the Golgi apparatus. This is achieved by the formation of [[transport vesicles]] that bud from the ER, which are then transported to the Golgi apparatus where they fuse and deliver their cargo. The proteins transported by COPII include both [[soluble proteins]] that reside in the lumen of the ER and [[transmembrane proteins]] that are embedded in the ER membrane. == Role in Disease == Mutations in the genes encoding COPII proteins have been linked to several human diseases, including [[Cranio-lenticulo-sutural dysplasia]] and [[Cole-Carpenter syndrome]]. These diseases are characterized by skeletal abnormalities, and are thought to result from defects in the transport of specific proteins that are important for bone development. == See Also == * [[Coat protein]] * [[Endoplasmic reticulum]] * [[Golgi apparatus]] * [[ER to Golgi transport]] * [[Secretory pathway]] * [[Transport vesicle]] * [[GTPase]] * [[Soluble protein]] * [[Transmembrane protein]] * [[Cranio-lenticulo-sutural dysplasia]] * [[Cole-Carpenter syndrome]] [[Category:Protein families]] [[Category:Cell biology]] [[Category:Membrane biology]] {{Cell-bio-stub}} <gallery> File:PDB_2gao_EBI.jpg|COPII File:CopIIcomplexbet1.png|COPII File:CopIIcomplexsedt5.png|COPII </gallery>
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