抗人 KIFBP 抗体对
01AB01503
Kinesin family binding protein (KIFBP), also known as KBP, is a protein characterized by tetratricopeptide repeat (TPR) motifs that adopts a solenoid-like, α-helical structure and binds to the motor domains of specific kinesins, such as KIF15 and KIF18A, through multivalent interactions involving distinct loops and helices. KIFBP inhibits kinesin motor activity by inducing allosteric changes and sterically blocking the microtubule-binding surface, thereby preventing microtubule attachment and ATPase activation, which is essential for proper axonal microtubule organization and neural development. Mutations in KIFBP are associated with Goldberg-Shprintzen syndrome, a rare congenital disorder characterized by intellectual disability, microcephaly, Hirschsprung disease, and cortical dysplasia, highlighting its critical role in neural development and axonal maintenance. Thus, KIFBP¡'s disruption leads to severe neurodevelopmental defects due to impaired regulation of kinesin-mediated microtubule dynamics.
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技术参数
- 靶点基因名
- KIFBP
- 基因别名
- KBP; KIAA1279; KIF1BP
- 蛋白别名
- kinesin family binding protein; KIFBP
- UniProt
- Q96EK5
- 物种
- Human
产品详情
- 靶点名称
- KIFBP
- 蛋白名称
- kinesin family binding protein
- 蛋白别名
- KIF-binding protein; KIF1-binding protein; Kinesin family binding protein
- 靶点基因名
- KIFBP
- 基因别名
- KBP; KIAA1279; KIF1BP
- UniProt
- Q96EK5
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Kinesin family binding protein (KIFBP), also known as KBP, is a protein characterized by tetratricopeptide repeat (TPR) motifs that adopts a solenoid-like, α-helical structure and binds to the motor domains of specific kinesins, such as KIF15 and KIF18A, through multivalent interactions involving distinct loops and helices. KIFBP inhibits kinesin motor activity by inducing allosteric changes and sterically blocking the microtubule-binding surface, thereby preventing microtubule attachment and ATPase activation, which is essential for proper axonal microtubule organization and neural development. Mutations in KIFBP are associated with Goldberg-Shprintzen syndrome, a rare congenital disorder characterized by intellectual disability, microcephaly, Hirschsprung disease, and cortical dysplasia, highlighting its critical role in neural development and axonal maintenance. Thus, KIFBP¡'s disruption leads to severe neurodevelopmental defects due to impaired regulation of kinesin-mediated microtubule dynamics.