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Szczepaska A, Olek K, Koodziejska K, Yu J, Ibrahim AT, Adamkiewicz L, Schroeder FC, Pokrzywa W, Turek M

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L.WilsonL

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& West, A

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Collagen electrophoresis can be used to study the effect of the variant but the absence of demonstration of abnormal collagen III produced by fibroblasts does not exclude the diagnosis

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Thus, it is still unknown whether there is an underlying deficit in mitochondrial respiratory function in these HepG2 citrindeficient cells, or whether the observed phenotype is merely a result of reduced cell number and mitochondrial density related to the citrin deficiency

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