Mitochondrial form and function in hair cells

TY - JOUR

T1 - Mitochondrial form and function in hair cells

AU - O'Sullivan, James D.B.

AU - Bullen, Anwen

AU - Mann, Zoë F.

N1 - Funding Information: The authors acknowledge the support of the Biotechnology & Biological Research Council (Z.F.M. BB/V006371/1). We also thank Professor Jonathan Ashmore, Professor Andy Forge, Professor Dan Jagger, Professor Jonathan Gale and Dr Lisa Nolan for critical evaluation and discussion on earlier versions of this article. Publisher Copyright: © 2022 The Author(s)

PY - 2023/2

Y1 - 2023/2

N2 - Hair cells (HCs) are specialised sensory receptors residing in the neurosensory epithelia of inner ear sense organs. The precise morphological and physiological properties of HCs allow us to perceive sound and interact with the world around us. Mitochondria play a significant role in normal HC function and are also intricately involved in HC death. They generate ATP essential for sustaining the activity of ion pumps, Ca2+ transporters and the integrity of the stereociliary bundle during transduction as well as regulating cytosolic calcium homoeostasis during synaptic transmission. Advances in imaging techniques have allowed us to study mitochondrial populations throughout the HC, and how they interact with other organelles. These analyses have identified distinct mitochondrial populations between the apical and basolateral portions of the HC, in which mitochondrial morphology appears determined by the physiological processes in the different cellular compartments. Studies in HCs across species show that ototoxic agents, ageing and noise damage directly impact mitochondrial structure and function resulting in HC death. Deciphering the molecular mechanisms underlying this mitochondrial sensitivity, and how their morphology relates to their function during HC death, requires that we first understand this relationship in the context of normal HC function.

AB - Hair cells (HCs) are specialised sensory receptors residing in the neurosensory epithelia of inner ear sense organs. The precise morphological and physiological properties of HCs allow us to perceive sound and interact with the world around us. Mitochondria play a significant role in normal HC function and are also intricately involved in HC death. They generate ATP essential for sustaining the activity of ion pumps, Ca2+ transporters and the integrity of the stereociliary bundle during transduction as well as regulating cytosolic calcium homoeostasis during synaptic transmission. Advances in imaging techniques have allowed us to study mitochondrial populations throughout the HC, and how they interact with other organelles. These analyses have identified distinct mitochondrial populations between the apical and basolateral portions of the HC, in which mitochondrial morphology appears determined by the physiological processes in the different cellular compartments. Studies in HCs across species show that ototoxic agents, ageing and noise damage directly impact mitochondrial structure and function resulting in HC death. Deciphering the molecular mechanisms underlying this mitochondrial sensitivity, and how their morphology relates to their function during HC death, requires that we first understand this relationship in the context of normal HC function.

KW - Ca2+buffering

KW - Development

KW - Hearing loss

KW - Metabolism

KW - Mitochondria

KW - Mitochondrial cristae

KW - Synaptic transmission oxidative stress

UR - http://www.scopus.com/inward/record.url?scp=85145671992&partnerID=8YFLogxK

U2 - 10.1016/j.heares.2022.108660

DO - 10.1016/j.heares.2022.108660

M3 - Review article

C2 - 36525891

AN - SCOPUS:85145671992

SN - 0378-5955

VL - 428

JO - Hearing Research

JF - Hearing Research

M1 - 108660

ER -