FURIN

This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. It encodes a type 1 membrane bound protease that is expressed in many tissues, including neuroendocrine, liver, gut, and brain. The encoded protein undergoes an initial autocatalytic processing event in the ER and then sorts to the trans-Golgi network through endosomes where a second autocatalytic event takes place and the catalytic activity is acquired. Like other members of this convertase family, the product of this gene specifically cleaves substrates at single or paired basic residues. Some of its substrates include proparathyroid hormone, transforming growth factor beta 1 precursor, proalbumin, pro-beta-secretase, membrane type-1 matrix metalloproteinase, beta subunit of pro-nerve growth factor and von Willebrand factor. It is thought to be one of the proteases responsible for the activation of HIV envelope glycoproteins gp160 and gp140, and may play a role in tumor progression. Unlike SARS-CoV and other coronaviruses, the spike protein of SARS-CoV-2 is thought to be uniquely cleaved by this protease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2020]

本基因编码亚tilisina样前体蛋白转换酶家族成员，该家族包括负责处理通过调节性或组成性分泌途径运输的蛋白质和肽前体的蛋白酶。它编码一种类型1的膜结合蛋白酶，在包括神经内分泌、肝脏、肠道和大脑在内的多种组织中表达。编码的蛋白在糙面内质网中经历初始的自催化加工事件，随后通过内体被分配到高尔基体跨膜区，在那里发生第二次自催化事件并获得催化活性。与其他此转换酶家族成员相似，本基因产物特异性地切割含单个或成对碱性残基的底物。其底物包括甲状旁腺素前体、转化生长因子β1前体、前白蛋白、前β-分泌酶、膜型1基质金属蛋白酶、前神经生长因子β亚基和冯·威勒布兰德因子。据信，它是负责激活HIV包膜糖蛋白gp160和gp140的蛋白酶之一，可能在肿瘤进展中发挥作用。与SARS-CoV和其他冠状病毒不同，SARS-CoV-2的刺突蛋白被认为是由这种蛋白酶特异性切割的。可变剪接导致多种转录本变体。[由RefSeq提供，2020年8月]