The Complement Regulator Susd4 Influences Nervous-System Function and Neuronal Morphology in Mice.
The Complement Regulator Susd4 Influences Nervous-System Function and Neuronal Morphology in Mice.
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补体调节剂 Susd4 影响小鼠的神经系统功能和神经元形态。
DOI：
10.1016/j.isci.2020.100957
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发表时间：
2020
期刊：
影响因子：
5.8
通讯作者：
Proia,RichardL
中科院分区：
文献类型：
--
作者：
Zhu,Hongling;Meissner,LauraE;Byrnes,Colleen;Tuymetova,Galina;Tifft,CynthiaJ;Proia,RichardL
TheSUSD4(Sushi domain-containing protein 4) gene encodes a complement inhibitor that is frequently deleted in 1q41q42 microdeletion syndrome, a multisystem congenital disorder that includes neurodevelopmental abnormalities. To understand SUSD4's role in the mammalian nervous system, we analyzedSusd4knockout (KO) mice.Susd4KO mice exhibited significant defects in motor performance and significantly higher levels of anxiety-like behaviors.Susd4KO brain had abnormal "hairy" basket cells surrounding Purkinje neurons within the cerebellum and significantly reduced dendritic spine density in hippocampal pyramidal neurons. Neurons and oligodendrocyte lineage cells of wild-type mice were found to expressSusd4mRNA. Protein expression of the complement component C1q was increased in the brains ofSusd4KO mice. Our data indicate that SUSD4 plays an important role in neuronal functions, possibly via the complement pathway, and thatSUSD4deletion may contribute to the nervous system abnormalities in patients with 1q41q42 deletions.
