Centromere Protein B (CENP-B)/着丝粒蛋白B

Centromere Protein B (CENP-B)

Diseases：
Scleroderma 

Centromere proteins are a group of proteins which form and/or mediate the function of centromeres, the central structures of chromosomes to which spindle fibers/microtubuli attach and pull the chromosomes apart in cell division. Currently, 9 centromere proteins are known and designated CENP-A to CENP-I. Most of the centromere proteins are targets of autoantibodies, the anti-centromere antibodies.

CENP-B is the most important of the centromeric autoantigens: it has a molecular weight of approx. 66 kDa and plays an important role in the formation of the centromeric chromatin. CENP-B antibodies are present in the sera of up to 80% of patients with CREST syndrome.

CENP-B antigen from DIARECT is produced in the baculovirus / insect cell expression system.

相关疾病：硬皮病/CREST综合征系统性硬化病（SSc）是一种结缔组织全身紊乱疾病，影响皮肤和内部器官，以外周动脉粥样硬化和内脏结构纤维化为特征。在皮肤和内脏的细胞外基质（主要是胶原）不同程度的积累，系统性硬化病常常和特异性自身抗体相关，尤其是着丝粒抗体和Scl-70抗体（DNA拓扑异构酶I的自身抗体）。其他类型的自身抗体，如抗PM/Scl抗体，认为是伴有肌炎、硬皮病和PM/Scl重叠综合症的另一亚类自身免疫病人的特征。硬皮病可能导致心、肺、肾和肠胃道的纤维化和功能衰退。着丝粒蛋白是形成或调节着丝粒功能的一类蛋白，染色体的中心结构附有纺锤丝和微管蛋白，而使得染色体在细胞分裂时得以分离。目前，已知9个着丝粒蛋白，为CENP-A到CENP-I形成、活性极其重要, 影响着染色体的稳定存在和正常分离。目前知道的着丝粒蛋白有9种，命名为CENP-A到CENP-I。大多数着丝粒蛋白是自身抗体和抗着丝粒抗体的靶点。CENP-B 的分子量接近于66kDa，在着丝粒染色质的形成中起到重要作用。CENP-B抗体在80%的CREST综合征患者的血清中都能检测到。

DIARECT生产的CENP-B抗原是在杆状病毒/昆虫表达系统中表达的。