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  1. No Access

    Article

    Biosynthesis of glycosaminoglycans: associated disorders and biochemical tests

    Glycosaminoglycans (GAG) are long, unbranched heteropolymers with repeating disaccharide units that make up the carbohydrate moiety of proteoglycans. Six distinct classes of GAGs are recognized. Their synthesi...

    Florin Sasarman, Catalina Maftei… in Journal of Inherited Metabolic Disease (2016)

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    Radioactive Labeling of Mitochondrial Translation Products in Cultured Cells

    The mammalian mitochondrial genome contains 37 genes, 13 of which encode polypeptide subunits in the enzyme complexes of the oxidative phosphorylation system. The other genes encode the rRNAs and tRNAs necessa...

    Florin Sasarman, Eric A. Shoubridge in Mitochondrial Disorders (2012)

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    Article

    Mutation in TACO1, encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

    Eric Shoubridge and colleagues report the identification of a mutation in the CCDC44 gene that is causal in a Leigh syndrome pedigree. The CCDC44 gene product, TACO1, is involved in mitochondrial translation and ...

    Woranontee Weraarpachai, Hana Antonicka, Florin Sasarman, Jürgen Seeger… in Nature Genetics (2009)

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    Oxidative Phosphorylation: Synthesis of Mitochondrially Encoded Proteins and Assembly of Individual Structural Subunits into Functional Holoenzyme Complexes

    The bulk of ATP consumed by various cellular processes in higher eukaryotes is normally produced by five multimeric protein complexes (I–V) embedded within the inner mitochondrial membrane, in a process known ...

    Scot C. Leary, Florin Sasarman in Mitochondrial DNA (2009)