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iPSC-derived motor neuron studies. (A) Western blot from motor neurons shows reduced levels of COQ7 in motor neurons derived from a COQ7 patient, proband from Family 2). (B) ELISA quantification of NFL protein release from iPSC-derived motor neurons. Motor neurons from COQ7 patients show significantly increased levels of cell supernatant NFL protein, indicating axonal degeneration (t-test, P < 0.001).
