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© Thieme Medical Publishers.Mucolipidosis II α/beta (MLII) is an autosomal recessive illness in which a gene mutation leads to improper focusing on of lysosomal enzymes with a finish result of accumulation of lysosomes within the mitochondria leading to a dysfunctional mitochondria. 1 Leigh syndrome (LS) is a rare progressive neurodegenerative disorder connected with dysfunctional mitochondria and oxidative phosphorylation. 4 Both disease processes usually presen
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