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Coatomer subunit beta 1 (COPB1) is a protein encoded by the COPB1 gene and acts as a core component of the coat protein complex I (COPI), a cytosolic complex mediating vesicular transport between the endoplasmic reticulum and the Golgi apparatus[1][2][3][5]. COPB1 binds dilysine motifs and participates in budding and fusion of non-clathrin-coated vesicles essential for retrograde trafficking of proteins and lipids, Golgi membrane dynamics, and compartmentalization within the early secretory pathway[1][2][5]. It also plays roles in lipid droplet homeostasis, autophagy, and proper degradation of specific proteins, being vital for cellular homeostasis and organelle function[1][5]. Disease mutations can cause congenital disorders such as Baralle-Macken syndrome and primary bone dysplasia[1]. As a ubiquitous and essential intracellular transport protein, COPB1 is not considered a direct therapeutic target, and no approved drugs or biomarkers are associated with it[1][5].
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