title: Distribution of transglutaminase 6 in the central nervous system of adult mice.
abstract: Our previous study identified a new form of spinocerebellar ataxia (SCA), in which mutations in the gene coding for transglutaminase 6 (TG6) were suggested to be causative. However, the data concerning cellular distribution of TG6 in the brain is still fragmentary. Therefore, we now report a comprehensive immunohistochemical examination of the expression profile of TG6 in adult mouse brain. TG6 was abundantly expressed in the septal region, basal ganglia, hypothalamus and brainstem. Notably, numerous TG6-positive neurons were found in the key brain regions involved in regulating locomotion activity, including the globus pallidus, subthalamic nucleus, substantia nigra, cerebellum, some precerebellar nuclei, and spinal motor neurons. Double immunostaining showed that the vast majority of TG6-positive neurons in the reticular nigra were GABAergic and those in the compact nigra were not dopaminergic. In addition, double staining for TG6 with either anti-NeuN or glial fibrillary acidic protein (GFAP) antibodies demonstrated exclusive NeuN-TG6 co-localization. This study presents a comprehensive overview of TG6 expression in the mouse brain, and provides insight for investigating the role of TG6 in the development of SCA.
authors.affiliation: Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410008, China.
authors.forename: Yu-Tao
authors.initials: YT
authors.lastname: Liu
authors.forename: Bei-Sha
authors.initials: BS
authors.lastname: Tang
authors.forename: Wei
authors.initials: W
authors.lastname: Lan
authors.forename: Ning-Ning
authors.initials: NN
authors.lastname: Song
authors.forename: Ying
authors.initials: Y
authors.lastname: Huang
authors.forename: Lei
authors.initials: L
authors.lastname: Zhang
authors.forename: Wen-Juan
authors.initials: WJ
authors.lastname: Guan
authors.forename: Yu-Ting
authors.initials: YT
authors.lastname: Shi
authors.forename: Lu
authors.initials: L
authors.lastname: Shen
authors.forename: Hong
authors.initials: H
authors.lastname: Jiang
authors.forename: Ji-Feng
authors.initials: JF
authors.lastname: Guo
authors.forename: Kun
authors.initials: K
authors.lastname: Xia
authors.forename: Yu-Qiang
authors.initials: YQ
authors.lastname: Ding
authors.forename: Jun-Ling
authors.initials: JL
authors.lastname: Wang
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002490:Central Nervous System / Q000166:cytology / Q000378:metabolism*; D005260:Female; D007124:Immunoenzyme Techniques; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D009474:Neurons / Q000166:cytology / Q000378:metabolism*; D011503:Transglutaminases / Q000378:metabolism*
chemical_list: C532876:TGM6 protein, human; D011503:Transglutaminases
keywords: brain; distribution; immunohistochemistry; spinocerebellar ataxia; transglutaminase 6