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Mitochondrial development plays a role in cardiomyocyte maturation. Therefore, this study investigated whether <jats:italic>Speg<\/jats:italic> deficiency ( \u2013 \/ \u2013 ) in CMs would result in mitochondrial abnormalities. <jats:italic>Speg<\/jats:italic> wild-type and <jats:italic>Speg<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> C57BL\/6 littermate mice were utilized for assessment of mitochondrial structure by transmission electron and confocal microscopies. Speg was expressed in the first and second heart fields at embryonic (<jats:italic>E<\/jats:italic>) day 7.5, prior to the expression of mitochondrial Na<jats:sup>+<\/jats:sup>\/Ca2<jats:sup>+<\/jats:sup>\/Li<jats:sup>+<\/jats:sup> exchanger (NCLX) at E8.5. Decreases in NCLX expression (E11.5) and the mitochondrial-to-nuclear DNA ratio (E13.5) were observed in <jats:italic>Speg<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> hearts. Imaging of E18.5 <jats:italic>Speg<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> hearts revealed abnormal mitochondrial cristae, corresponding with decreased ATP production in cells fed glucose or palmitate, increased levels of mitochondrial superoxide and depolarization of mitochondrial membrane potential. Interestingly, phosphorylated (<jats:italic>p<\/jats:italic>) PGC-1\u03b1, a key mediator of mitochondrial development, was significantly reduced in <jats:italic>Speg<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> hearts during screening for targeted genes. Besides Z-line expression, Speg partially co-localized with PGC-1\u03b1 in the sarcomeric region and was found in the same complex by co-immunoprecipitation. Overexpression of a Speg internal serine\/threonine kinase domain in <jats:italic>Speg<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> CMs promoted translocation of pPGC-1\u03b1 into the nucleus, and restored ATP production that was abolished by siRNA-mediated silencing of PGC-1\u03b1. Our results demonstrate a critical role of Speg in mitochondrial development and energy metabolism in CMs, mediated in part by phosphorylation of PGC-1\u03b1.<\/jats:p>","DOI":"10.1007\/s00395-023-01029-7","type":"journal-article","created":{"date-parts":[[2023,12,26]],"date-time":"2023-12-26T05:05:21Z","timestamp":1703567121000},"page":"151-168","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Striated preferentially expressed gene deficiency leads to mitochondrial dysfunction in developing cardiomyocytes"],"prefix":"10.1007","volume":"119","author":[{"given":"Gu","family":"Li","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"He","family":"Huang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanshuang","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang","family":"Shu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narae","family":"Hwang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qifei","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rose","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hilaire C.","family":"Lam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William M.","family":"Oldham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Souheil","family":"EI-Chemaly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pankaj B.","family":"Agrawal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Tian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2904-4413","authenticated-orcid":false,"given":"Xiaoli","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark A.","family":"Perrella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,12,26]]},"reference":[{"key":"1029_CR1","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1016\/j.ajhg.2014.07.004","volume":"95","author":"PB Agrawal","year":"2014","unstructured":"Agrawal PB, Pierson CR, Joshi M, Liu X, Ravenscroft G, Moghadaszadeh B, Talabere T, Viola M, Swanson LC, Haliloglu G, Talim B, Yau KS, Allcock RJ, Laing NG, Perrella MA, Beggs AH (2014) SPEG interacts with myotubularin, and its deficiency causes centronuclear myopathy with dilated cardiomyopathy. 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