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Here we report a spontaneous Novel Ataxic Phenotype in mice. Ataxia findings were evident at post\u2010natal day 11 in NAP mice and rapidly worsened, resulting in preweaning lethality. Using genome sequencing and genome\u2010wide mapping, we identified a 3\u2032 donor splice variant in exon 14 of\n                    <jats:italic>Myo5a<\/jats:italic>\n                    , encoding an actin\u2010based motor protein. The variant in\n                    <jats:italic>Myo5a<\/jats:italic>\n                    (c.1752g&gt;a) excises exon 14 and ablates MYO5A protein expression, which is implicated in intracellular transport and Griscelli syndrome type I in humans. NAP mice displayed expansion of PAX6\u2010positive cells in the external granule layer of the cerebellum, and mass spectrometry analysis of cerebellar extracts uncovered differentially abundant proteins involved in short\u2010range organelle transport, and specifically proteins implicated with early endosomes. Using cerebellar lysates and primary neurons, we provide evidence for an interaction between MYO5A and ANKFY1, a known effector for the endosomal protein, RAB5A. We also found neurons from NAP mice had elongated mitochondria, linking MYO5A to mitochondrial homeostasis. This allele provides new insight into\n                    <jats:italic>Myo5a<\/jats:italic>\n                    function in developmental neuropathology.\n                  <\/jats:p>","DOI":"10.1096\/fj.202402274r","type":"journal-article","created":{"date-parts":[[2025,3,11]],"date-time":"2025-03-11T12:01:44Z","timestamp":1741694504000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A novel, rapidly progressive ataxia due to a spontaneous\n                    <i>Myo5a<\/i>\n                    mutation in mice impairs transport proteins and alters mitochondria"],"prefix":"10.1096","volume":"39","author":[{"given":"Alexander M.","family":"Telenson","sequence":"first","affiliation":[{"name":"Center for Genetic Medicine Feinberg School of Medicine, Northwestern University  Chicago Illinois USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryan 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