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Research explains the new function of Cenpj, a microcephaly gene in human

Updated: 2019-03-14

<>Autosomal recessive primary microcephaly (MCPH) is a neurodevelopmental disorder showing the major symptoms of reduction of circumference of the head, brain volume and cortex thickness with normal brain architecture in birth, accompanied by mental development disorders, emotional disorders and other symptoms, which seriously affect the normal life of patients. Most of the mutations leading to microcephaly are genes encoding centrosomal proteins. However, the cellular and molecular mechanism for these centrosomal proteins regulation in cortical development remains unclear.

The research work from WANG Xiaoqun’s group at the Institute of Biophysics (IBP) entitled “Cenpj regulates cilia disassembly and neurogenesis in the developing mouse cortex” was published in The Journal of Neuroscience on Mar. 13th, 2019. It describes that Cenpj, a microcephalygene encoding protein regulates cilia disassembly and neurogenesis in the developing mouse cortex.

The researchers used conditional Cenpj deletion mice and found that neural progenitor cells exhibited long primary cilia and abnormal cilium appendages. The defective cilium disassembly caused by Cenpj depletion led to reduced cell proliferation, uncompleted cell division, cell apoptosis, and microcephaly in mice. Cenpj also regulated cilium structure of adult neural stem cells and adult neurogenesis in mice. Additionally, their results illustrated that Cenpj regulated cilia disassembly and neurogenesis through Kif2a, indicating that primary cilia dynamic plays a crucial role in neural progenitor cell mitosis and adult neurogenesis.

This work was supported by grants from the Strategic Priority Research Program of the Chinese Academy of Sciences, Ministry of Science and Technology of China, the National Natural Science Foundation of China. The Center for Biological Imaging, Institute of Biophysics, provided the technical support of EM sample preparation and EM imaging.

 

Fig 1. The model of Cenpj function in neurogenesis in the developingmouse cortex

(Imaged by Dr: Wang Xiaoqun’s group)


Article link: http://www.jneurosci.org/content/39/11/1994


Contact: Wang Xiaoqun, Ph.D.
Principal Investigator 

Institute of biophysics, Chinese Academy of Sciences
Beijing100101, China
Tel: (86)-10-64887994 

Email: xiaoqunwang@ibp.ac.cn

(Reported by Dr. Wang Xiaoqun’s  group)

 

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