Planar cell polarity defects and hearing loss in sperm-associated antigen 6 ( Spag6 )-deficient mice

encodes an axoneme central apparatus protein that is required for normal flagellar and cilia motility. Recent findings suggest that also plays a role in ciliogenesis, orientation of cilia basal feet, and planar polarity. Sensory cells of the inner ear display unique structural features that underlie...

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Bibliographic Details
Published in:American Journal of Physiology: Cell Physiology 2021-01, Vol.320 (1), p.C132-C141, Article ajpcell.00166.2020
Main Authors: Li, Xiaofei, Zhang, Daogong, Xu, Lei, Han, Yuechen, Liu, Wenwen, Li, Wei, Fan, Zhaomin, Costanzo, Richard M, Strauss Iii, Jerome F, Zhang, Zhibing, Wang, Haibo
Format: Article
Language:English
Subjects:
Animals
Basal bodies
Cell Polarity
Cell size
Cilia
Female
Flagella
Frizzled protein
Frizzled Receptors - metabolism
Hair Cells, Auditory, Inner - metabolism
Hair Cells, Auditory, Inner - ultrastructure
Hearing
Hearing loss
Hearing Loss - genetics
Hearing Loss - metabolism
Hearing Loss - pathology
Hearing Loss - physiopathology
Inner ear
Male
Mice
Mice, 129 Strain
Mice, Inbred C57BL
Mice, Knockout
Microtubule Proteins - deficiency
Microtubule Proteins - genetics
Microtubules - metabolism
Microtubules - ultrastructure
Pattern formation
Polarity
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Description
Summary:encodes an axoneme central apparatus protein that is required for normal flagellar and cilia motility. Recent findings suggest that also plays a role in ciliogenesis, orientation of cilia basal feet, and planar polarity. Sensory cells of the inner ear display unique structural features that underlie their mechanosensitivity. They represent a distinctive form of cellular polarity, known as planar cell polarity (PCP). However, a role for in the inner ear has not yet been explored. In the present study, the function of in the inner ear was examined using -deficient mice. Our results demonstrate hearing loss in the mutants, associated with abnormalities in cellular patterning, cell shape, stereocilia bundles, and basal bodies, as well as abnormally distributed Frizzled class receptor 6 (FZD6), suggesting that participates in PCP regulation. Moreover, we found that the subapical microtubule meshwork was disrupted. Our observations suggest new functions for in hearing and PCP in the inner ear.
ISSN:0363-6143
1522-1563
DOI:10.1152/ajpcell.00166.2020