# Developmental Expression of Membrane Pumps and Ion Channels in Human Vestibular Endolymph Homeostasis

**Authors:** Edward S. A. van Beelen, Wouter H. van der Valk, John C. M. J. de Groot, Peter Paul G. van Benthem, Heiko Locher

PMC · DOI: 10.1002/dneu.70010 · Developmental Neurobiology · 2026-01-25

## TL;DR

This study maps the development of ion pumps and channels in the human vestibular system, revealing their roles in endolymph homeostasis during fetal growth.

## Contribution

The study provides the first detailed spatiotemporal expression profiles of ion transporters in the developing human vestibular system.

## Key findings

- ATP1A1 and ATP1B2 co-localize at the basolateral membranes of dark cells in the developing human vestibular system.
- BSND and SLC12A2 are expressed in transitional and dark cells starting from fetal week 10.
- KCNQ1 and GJA1/GJB2/GJB6 are expressed in transitional and dark cells from fetal week 14.

## Abstract

The expression patterns of key membrane pumps and ion channels involved in endolymph cycling have been studied in the rodent inner ear and the developing and adult human cochlea. However, little is known about their expression during the development of the human vestibular system. In this study, we provide a comprehensive overview of expression profiles of ion pumps, cotransporters, and exchangers in the developing human utricle and ampullae from fetal week (FW) 8 to 17. Immunohistochemistry analysis revealed that ATP1A1 and ATP1B2 co‐localize at the basolateral membranes of dark cells. In addition, BSND expression was observed in transitional cells and dark cells in both the ampulla and utricle from FW10. We further characterized the expression of gap junction proteins (GJA1, GJB2, and GJB6) and found that KCNQ1 was expressed by transitional cells and dark cells starting from FW14. SLC12A2 immunostaining was detected in dark cells around FW10. Lastly, we investigated the spatiotemporal expression of pendrin. These detailed observations of protein expression during human inner ear development enhance our understanding of endolymph homeostasis.

## Linked entities

- **Genes:** ATP1A1 (ATPase Na+/K+ transporting subunit alpha 1) [NCBI Gene 476], ATP1B2 (ATPase Na+/K+ transporting subunit beta 2) [NCBI Gene 482], BSND (barttin CLCNK type accessory subunit beta) [NCBI Gene 7809], KCNQ1 (potassium voltage-gated channel subfamily Q member 1) [NCBI Gene 3784], SLC12A2 (solute carrier family 12 member 2) [NCBI Gene 6558], GJA1 (gap junction protein alpha 1) [NCBI Gene 2697], GJB2 (gap junction protein beta 2) [NCBI Gene 2706], GJB6 (gap junction protein beta 6) [NCBI Gene 10804]

## Full-text entities

- **Genes:** KCNQ1 (potassium voltage-gated channel subfamily Q member 1) [NCBI Gene 3784] {aka ATFB1, ATFB3, JLNS1, KCNA8, KCNA9, KVLQT1}, GJB6 (gap junction protein beta 6) [NCBI Gene 10804] {aka CX30, DFNA3, DFNA3B, DFNB1B, ECTD2, ED2}, ATP1A1 (ATPase Na+/K+ transporting subunit alpha 1) [NCBI Gene 476] {aka CMT2DD, HOMGSMR2}, ATP1B2 (ATPase Na+/K+ transporting subunit beta 2) [NCBI Gene 482] {aka AMOG}, GJA1 (gap junction protein alpha 1) [NCBI Gene 2697] {aka AVSD3, CMDR, CX43, EKVP, EKVP3, GJAL}, GJB2 (gap junction protein beta 2) [NCBI Gene 2706] {aka BAPS, CX26, DFNA3, DFNA3A, DFNB1, DFNB1A}, BSND (barttin CLCNK type accessory subunit beta) [NCBI Gene 7809] {aka BART, DFNB73}, SLC12A2 (solute carrier family 12 member 2) [NCBI Gene 6558] {aka BSC, BSC-2, BSC2, CCC1, KILQS, NKCC1}, SLC26A4 (solute carrier family 26 member 4) [NCBI Gene 5172] {aka DFNB4, EVA, PDS, TDH2B}
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

_Full body text omitted from this summary view._ Fetch the complete paper as Markdown: https://tomesphere.com/paper/PMC12832121/full.md

## Figures

8 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12832121/full.md

## References

47 references — full list in the complete paper: https://tomesphere.com/paper/PMC12832121/full.md

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Source: https://tomesphere.com/paper/PMC12832121