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Protein concentration was measured with BCA protein assay reagent (No

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elegans knockout model devoid of the entire coding sequence of the BBOX1 ortholog gbh-1 (ko) , and strains bearing patient-derived BBOX1 variants, including gbh-1(G283R) , gbh-1(G247Vfs*6) , and gbh-1(D72G) , which are orthologous to the human BBOX1 variants

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