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Abstract SIL1, a nucleotide exchange factor for the endoplasmic reticulum chaperone BiP, primarily binds nascent polypeptides to facilitate proper protein folding. It is a causative gene of Marinesco-Sjögren syndrome (MSS). Previous studies have indicated that SIL1 dysfunction disrupts the migration and positioning of cortical neurons during development, thereby impairing the structural establishment of the early neocortex.
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