Journal article
Lmx1a is essential for marginal cell differentiation and stria vascularis formation
Frontiers in cell and developmental biology, Vol.13, 1537505
03/05/2025
DOI: 10.3389/fcell.2025.1537505
PMCID: PMC11920146
PMID: 40109362
Abstract
The transcription factor Lmx1a is widely expressed during early inner ear development, and mice lacking Lmx1a expression exhibit fusion of cochlear and vestibular hair cells and fail to form the ductus reuniens and the endolymphatic sac. Lmx1a dreher ( Lmx1a dr/dr ), a recessive null mutation, results in non-functional Lmx1a expression, which expands from the outer sulcus to the stria vascularis and Reissner’s membrane. In the absence of Lmx1a , we observe a lack of proteins specific to the stria vascularis, such as BSND and KCNQ1 in marginal cells and CD44 in intermediate cells. Further analysis of the superficial epithelial cell layer at the expected stria vascularis location shows that the future intermediate cells migrate during embryonic development but subsequently disappear. Using antibodies against pendrin ( Slc26a4 ) in Lmx1a knockout (KO) mice, we observe an expansion of pendrin expression across the stria vascularis and Reissner’s membrane. Moreover, in the absence of Lmx1a expression, no endocochlear potential is observed. These findings highlight the critical role of Lmx1a in inner ear development, particularly in the differentiation of cochlear and vestibular structures, the recruitment of pigment cells, and the expression of proteins essential for hearing and balance.
Details
- Title: Subtitle
- Lmx1a is essential for marginal cell differentiation and stria vascularis formation
- Creators
- Justine M. Renauld - Creighton UniversityIgor Y. Iskusnykh - University of Tennessee Health Science CenterEbenezer N. YamoahRichard J. H. Smith - University of IowaCorentin Affortit - University of IowaDavid Z. He - Creighton UniversityHuizhan Liu - Creighton UniversityDavid Nichols - Creighton UniversityJudith Bouma - Creighton UniversityMahesh K. Nayak - Creighton UniversityXin Weng - Chinese University of Hong KongTianli Qin - Chinese University of Hong KongMai Har Sham - Chinese University of Hong KongVictor V. Chizhikov - University of Tennessee Health Science CenterBernd Fritzsch - University of Nebraska Medical Center
- Resource Type
- Journal article
- Publication Details
- Frontiers in cell and developmental biology, Vol.13, 1537505
- DOI
- 10.3389/fcell.2025.1537505
- PMID
- 40109362
- PMCID
- PMC11920146
- NLM abbreviation
- Front Cell Dev Biol
- ISSN
- 2296-634X
- eISSN
- 2296-634X
- Publisher
- FRONTIERS MEDIA SA
- Grant note
- National Institute for Health and Care Research10.13039/501100000272
The authors thank Garret Soukup and Marcia Pierce for their help in designing and preparing primers, Ian Jackson for the DCT plasmid, and Andreas Kispert for the Tbx18 plasmid.
- Language
- English
- Date published
- 03/05/2025
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Biology; Craniofacial Anomalies Research Center; Otolaryngology; Internal Medicine
- Record Identifier
- 9984799686802771
Metrics
62 Record Views