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Review
. 2019 Jun;67(6):429-433.
doi: 10.1007/s00106-019-0662-2.

Why study inner ear hair cell mitochondria?

Affiliations
Review

Why study inner ear hair cell mitochondria?

J Lesus et al. HNO. 2019 Jun.

Abstract

In several systems of the body (muscle, liver, nerves), new studies have examined the internal structure of mitochondria and brought to light striking new findings about how mitochondria are constructed and how their structure affects cell function. In the inner ear field, however, we have little structural knowledge about hair cell and supporting cell mitochondria, and virtually none about mitochondrial subtypes or how they function in health and disease. The need for such knowledge is discussed in this short review.

In anderen Systemen (Muskeln, Leber, Nerven) haben neue Studien die innere Struktur der Mitochondrien untersucht und aufschlussreiche neue Erkenntnisse daruber gewonnen, wie Mitochondrien aufgebaut sind und wie ihre Struktur die Zellfunktion beeinflusst. Im Innenohrbereich haben wir jedoch wenig strukturelles Wissen über Mitochondrien von Haarzellen und Stützzellen, und praktisch kein Wissen über mitochondriale Subtypen oder deren Funktion bei Gesundheit und Krankheit. Der Bedarf an solchem Wissen wird in diesem kurzen Überblick diskutiert.

Keywords: Cochlea; Deafness; Ototoxic aminoglycoside antibiotics; Vestibular disorders; mtDNA.

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Conflict of interest statement

Conflict of Interest

J. Lesus, K. Arias, J. Kulaga, S. Sobkiv, A. Patel, V. Babu, A.Kambalyal, M. Patel, F. Padron, P. Mozaffari, A. Jayakumar, L. Ghatalah, N. Laban, R. Bahari, G. Perkins, A. Lysakowski state that there are no conflicts of interest.

Figures

Figure 1.
Figure 1.
A schematic of one crista from a mitochondrion, viewed en face. This is a lamellar crista, one of many found in a mitochondrion, and it has several crista junctions (CJ) extending from it to the inner mitochondrial membrane (IMM), which sits inside the outer mitochondrial membrane (OMM). Note that there are more CJs on the right side than on the left side of the crista, which represents the finding that sometimes cellular elements on one side of a mitochondrion, such as synapses, cuticular plates, or other cellular organelles, have more CJs oriented toward them than away from them.
Figure 2.
Figure 2.
Three-dimensional reconstruction of a hair cell mitochondrion adjacent to a striated organelle [44] in a vestibular hair cell. A. A section taken from an electron microscopic tomogram, showing part of a striated organelle (SO) and a crista junction (CJ), the junction of the crista with the inner mitochondrial membrane. B. Same section as in A, but with the reconstructed cristae superimposed on the tomogram and yellow lines indicating the position of the SO. C. The full model of mitochondrion, the SO and the outer mitochondrial membrane (OMM). The CJs are shown intersecting with the inner membrane. D. The same mitochondrion viewed from the top, compared to C, looking through the SO elements at the CJs, which are represented as holes in the inner mitochondrial membrane.

References

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