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Zhang Q, Zhang B, Lim NKH, et al. Hyccin/FAM126A deficiency reduces glial enrichment and axonal sheath, which are rescued by overexpression of a plasma membrane-targeting PI4KIIIα in Drosophila. Biochem Biophys Res Commun. 2021;589:71-77doi: 10.1016/j.bbrc.2021.11.106.
Zhang, Q., Zhang, B., Lim, N. K. H., Zhang, X., Meng, S., Nyengaard, J. R., Huang, F., & Wang, W. A. (2022). Hyccin/FAM126A deficiency reduces glial enrichment and axonal sheath, which are rescued by overexpression of a plasma membrane-targeting PI4KIIIα in Drosophila. Biochemical and biophysical research communications, 58971-77. https://doi.org/10.1016/j.bbrc.2021.11.106
Zhang, Qichao, et al. "Hyccin/FAM126A deficiency reduces glial enrichment and axonal sheath, which are rescued by overexpression of a plasma membrane-targeting PI4KIIIα in Drosophila." Biochemical and biophysical research communications vol. 589 (2022): 71-77. doi: https://doi.org/10.1016/j.bbrc.2021.11.106
Zhang Q, Zhang B, Lim NKH, Zhang X, Meng S, Nyengaard JR, Huang F, Wang WA. Hyccin/FAM126A deficiency reduces glial enrichment and axonal sheath, which are rescued by overexpression of a plasma membrane-targeting PI4KIIIα in Drosophila. Biochem Biophys Res Commun. 2022 Jan 22;589:71-77. doi: 10.1016/j.bbrc.2021.11.106. Epub 2021 Dec 03. PMID: 34894559.
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Biochem Biophys Res Commun. 2022 Jan 22;589:71-77. doi: 10.1016/j.bbrc.2021.11.106. Epub 2021 Dec 03.

Hyccin/FAM126A deficiency reduces glial enrichment and axonal sheath, which are rescued by overexpression of a plasma membrane-targeting PI4KIIIα in Drosophila.

Biochemical and biophysical research communications

Qichao Zhang, Baozhu Zhang, Nastasia K H Lim, Xiao Zhang, Shiquan Meng, Jens R Nyengaard, Fude Huang, Wen-An Wang

Affiliations

  1. Sino-Danish College, University of Chinese Academy of Sciences, Beijing, 100190, China; Sino-Danish Center for Education and Research, Beijing, 100190, China; Shanghai Advanced Research Institute, University of Chinese Academy of Sciences, Chinese Academy of Sciences, Shanghai, 201210, China; Department of Clinical Medicine, Core Center for Molecular Morphology, Section for Stereology and Microscopy, Aarhus University, Aarhus, 8200, Denmark.
  2. Institute of Neuroscience and State Key Laboratory of Neuroscience, Shanghai Institute of Biological Sciences, Chinese Academy of Sciences, Shanghai, 200031, China.
  3. Shanghai Advanced Research Institute, University of Chinese Academy of Sciences, Chinese Academy of Sciences, Shanghai, 201210, China; Nuo-beta Pharmaceutical Technology (Shanghai) Co. Ltd., Shanghai, 201210, China; Shanghai Institute of Materia Medica, University of Chinese Academy of Sciences, Chinese Academy of Sciences, Shanghai, 201203, China.
  4. Shanghai Advanced Research Institute, University of Chinese Academy of Sciences, Chinese Academy of Sciences, Shanghai, 201210, China; Laboratory of Molecular Neurobiology, School of Life Sciences, Shanghai University, Shanghai, 200072, China.
  5. Sino-Danish College, University of Chinese Academy of Sciences, Beijing, 100190, China; Sino-Danish Center for Education and Research, Beijing, 100190, China; Department of Clinical Medicine, Core Center for Molecular Morphology, Section for Stereology and Microscopy, Aarhus University, Aarhus, 8200, Denmark.
  6. Sino-Danish College, University of Chinese Academy of Sciences, Beijing, 100190, China; Sino-Danish Center for Education and Research, Beijing, 100190, China; Shanghai Advanced Research Institute, University of Chinese Academy of Sciences, Chinese Academy of Sciences, Shanghai, 201210, China; Nuo-beta Pharmaceutical Technology (Shanghai) Co. Ltd., Shanghai, 201210, China. Electronic address: [email protected].
  7. Department of Neurology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, 200092, China; Department of Neurology, Xinhua Hospital Chongming Branch, Shanghai 202150, China. Electronic address: [email protected].

PMID: 34894559 DOI: 10.1016/j.bbrc.2021.11.106

Abstract

Hyccin/FAM126A mutations are linked to hypomyelination and congenital cataract disease (HCC), but whether and how Hyccin/FAM126A deficiency causes hypomyelination remains undetermined. This study shows Hyccin/FAM126A expression was necessary for the expression of other components of the PI4KIIIα complex in Drosophila. Knockdown of Hyccin/FAM126A in glia reduced the enrichment of glial cells, disrupted axonal sheaths and visual ability in the visual system, and these defects could be fully rescued by overexpressing either human FAM126A or FAM126B, and partially rescued by overexpressing a plasma membrane-targeting recombinant mouse PI4KIIIα. Additionally, PI4KIIIα knockdown in glia phenocopied Hyccin/FAM126A knockdown, and this was partially rescued by overexpressing the recombinant PI4KIIIα, but not human FAM126A or FAM126B. This study establishes an animal model of HCC and indicates that Hyccin/FAM126A plays an essential role in glial enrichment and axonal sheath in a cell-autonomous manner in the visual system via controlling the expression and stabilization of the PI4KIIIα complex at the plasma membrane.

Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

Keywords: FAM126A; FAM126B; Glia; Hyccin; PI4KIIIα; Phosphatidylinositols; TTC7

Conflict of interest statement

Declaration of competing interest All authors declare no competing interests.

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