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While the unique and redundant functions of\n Lmx1a\/b<\/jats:italic>\n in the inner ear are well established, their contribution to the development of the cochlear nuclei, which process and relay auditory information to the brain, is poorly understood. Since cochlear nuclei maturate postnatally, here we analyzed\n Lmx1a<\/jats:italic>\n \n +\/\u2212<\/jats:italic>\n <\/jats:sup>\n ;Lmx1b<\/jats:italic>\n \n +\/\u2212<\/jats:italic>\n <\/jats:sup>\n ,\n Lmx1a<\/jats:italic>\n \n \u2212\/\u2212<\/jats:italic>\n <\/jats:sup>\n , and\n Lmx1a<\/jats:italic>\n \n \u2212\/\u2212<\/jats:italic>\n <\/jats:sup>\n ;Lmx1b<\/jats:italic>\n \n +\/\u2212<\/jats:italic>\n <\/jats:sup>\n mice that survive past birth. Loss of\n Lmx1a<\/jats:italic>\n reduced distinct populations of excitatory neurons in dorsal (DCN) and ventral (VCN) cochlear nuclei and their innervation from the inner ear. Additional loss of one\n Lmx1b<\/jats:italic>\n copy made\n Lmx1a<\/jats:italic>\n \n \u2212\/\u2212<\/jats:italic>\n <\/jats:sup>\n phenotypes more severe, revealing that\n Lmx1b<\/jats:italic>\n acts redundantly with\n Lmx1a<\/jats:italic>\n . Unlike\n Lmx1a<\/jats:italic>\n \n \u2212\/\u2212<\/jats:italic>\n <\/jats:sup>\n mice, excitatory neurons were not affected in\n Lmx1a<\/jats:italic>\n \n +\/\u2212<\/jats:italic>\n <\/jats:sup>\n ;Lmx1b<\/jats:italic>\n \n +\/\u2212<\/jats:italic>\n <\/jats:sup>\n mice. Thus, while cochlear nuclei are sensitive to\n Lmx1a\/b<\/jats:italic>\n gene dosage, these genes are not completely equivalent, and\n Lmx1a<\/jats:italic>\n has a more profound role in cochlear nuclei development.\n Lmx1a<\/jats:italic>\n \n \u2212\/\u2212<\/jats:italic>\n <\/jats:sup>\n and especially\n Lmx1a<\/jats:italic>\n \n \u2212\/\u2212<\/jats:italic>\n <\/jats:sup>\n ;Lmx1b<\/jats:italic>\n +\/\u2212<\/jats:sup>\n embryos had fewer Atoh1+\u2009progenitors that produce excitatory neurons of the cochlear nuclei, and reduced\n Bmp6<\/jats:italic>\n expression in the roof plate, the signaling center that induces these progenitors via Bmp signaling. We found that\n Lmx1a<\/jats:italic>\n is the primary regulator of\n Bmp6<\/jats:italic>\n , whereas\n Lmx1b<\/jats:italic>\n contributes only in the absence of\n Lmx1a<\/jats:italic>\n . Thus,\n Lmx1a<\/jats:italic>\n plays a major role in the formation of the mature structure and connectivity of both the DCV and VCN, and\n Lmx1b<\/jats:italic>\n acts redundantly to\n Lmx1a<\/jats:italic>\n but only partially compensates for\n Lmx1a<\/jats:italic>\n loss.\n <\/jats:p>","DOI":"10.1007\/s00441-026-04064-7","type":"journal-article","created":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T02:30:25Z","timestamp":1776393025000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["An Lmx1a\/b allelic series reveals the role of Lmx1 genes in cochlear nuclei development"],"prefix":"10.1007","volume":"404","author":[{"given":"Igor Y.","family":"Iskusnykh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernd","family":"Fritzsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ebenezer N.","family":"Yamoah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ekaterina Y.","family":"Steshina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor V.","family":"Chizhikov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,17]]},"reference":[{"key":"4064_CR1","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1038\/9189","volume":"2","author":"J Alder","year":"1999","unstructured":"Alder J, Lee KJ, Jessell TM, Hatten ME (1999) Generation of cerebellar granule neurons in vivo by transplantation of BMP-treated neural progenitor cells. 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