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T1  - Ketogenic diet attenuates hepatopathy in mouse model of respiratory chain complex III deficiency caused by a Bcs1l mutation 
SN  -  /  
UR  - http://hdl.handle.net/10138/195631 
T3  -  
A1  - Purhonen, Janne; Rajendran, Jayasimman; Morgelin, Matthias; Uusi-Rauva, Kristiina; Katayama, Shintaro; Krjutskov, Kaarel; Einarsdottir, Elisabet; Velagapudi, Vidya; Kere, Juha; Jauhiainen, Matti; Fellman, Vineta; Kallijarvi, Jukka 
A2  -  
PB  -  
Y1  - 2017 
LA  - eng 
AB  - Mitochondrial disorders are among the most prevalent inborn errors of metabolism but largely lack treatments and have poor outcomes. High-fat, low-carbohydrate ketogenic diets (KDs) have shown beneficial effects in mouse models of mitochondrial myopathies, with induction of mitochondrial biogenesis as the suggested main mechanism. We fed KD to mice with respiratory chain complex III (CIII) deficiency and progressive hepatopathy due to mutated BCS1L, a CIII assembly factor. The mutant mice became... 
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KW  - PROGENITOR-CELL EXPANSION; LOW-CARBOHYDRATE; GRACILE SYNDROME; GENE-EXPRESSION; IRON-OVERLOAD; MITOCHONDRIAL; LIVER; MACROPHAGES; MICE; DISEASE; 3111 Biomedicine 
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