Early detection of elevated lactate levels in a mitochondrial disease model using chemical exchange saturation transfer (CEST) and magnetic resonance spectroscopy (MRS) with 7T MR imaging
To the Editor,
We appreciate the letter in response to our article . Our report revealed that chemical exchange saturation transfer (CEST) and magnetic resonance spectroscopy (MRS) using 7T magnetic resonance imaging (MRI) could detect lactate level elevation prior to the formation of cerebral morphological abnormalities in Ndufs4 knockout (KO) mice, a mouse model of Leigh syndrome .
The letter claimed that Ndufs4 KO mice investigated in the present study do not represent an animal model of Leigh syndrome. We performed MRI and CEST in the mice when they were 6 weeks of age. They did not show any lesions in T2-weighted MRI (T2WI), but at around 8 weeks of age they manifested Leigh syndrome features with 100% penetrance. Ndufs4 KO mice that we used in this study were produced and well established as a model of Leigh syndrome by the Palmiter Laboratory at the University of Washington [2, 3]. A model of Leigh syndrome is characterized by failure to thrive, generalized hypotonia,...
KeywordsNdufs4 knockout Leigh syndrome Chemical exchange saturation transfer Magnetic resonance spectroscopy
This work was supported by Grants-in-Aid for Scientific Research (Kakenhi, Nos. 16K090170 and 15K21774) from the Japan Society for the Promotion of Science (JSPS).
Compliance with ethical standards
Conflicts of interest
The authors have no conflicts of interest to report.
All applicable international, national, and/or institutional guidelines for the care and use of animals were followed. This article does not contain any studies with human participants performed by any of the authors.
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