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人 COPB2 抗体对
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人 COPB2 抗体对

01AB03095

Coat protein complex I subunit beta 2 (COPB2) is a cytosolic subunit of the COPI coatomer complex, essential for retrograde vesicular trafficking from the Golgi to the endoplasmic reticulum and intra-Golgi transport. Recruited by ARF GTPases, it recognizes dilysine motifs on cargo proteins to facilitate their retrieval and maintain Golgi structure. COPB2 is also a receptor for activated PKC-epsilon. Pathogenic biallelic variants in COPB2 cause primary autosomal recessive microcephaly 19 (MCPH19) and are associated with developmental delay and osteoporosis, underscoring its critical requirement for neurodevelopment and skeletal homeostasis.

Human
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技术参数

靶点基因名
COPB2
蛋白别名
coat protein complex I subunit beta 2; COPB2
UniProt
P35606
物种
Human

产品详情

靶点名称
COPB2
蛋白名称
coat protein complex I subunit beta 2
蛋白别名
Coatomer subunit beta'; Beta'-coat protein; Beta'-COP; p102);COPI coat complex subunit beta 2
靶点基因名
COPB2
UniProt
P35606
来源物种
Homo sapiens (Human)
物种反应性
Human

背景介绍

Coat protein complex I subunit beta 2 (COPB2) is a cytosolic subunit of the COPI coatomer complex, essential for retrograde vesicular trafficking from the Golgi to the endoplasmic reticulum and intra-Golgi transport. Recruited by ARF GTPases, it recognizes dilysine motifs on cargo proteins to facilitate their retrieval and maintain Golgi structure. COPB2 is also a receptor for activated PKC-epsilon. Pathogenic biallelic variants in COPB2 cause primary autosomal recessive microcephaly 19 (MCPH19) and are associated with developmental delay and osteoporosis, underscoring its critical requirement for neurodevelopment and skeletal homeostasis.