The SEC23A gene is mapped to chromosome 14q21.1. The gene encodes a member of the SEC23 subfamily of the SEC23/SEC24 family. The SEC23A protein is found in the ribosomal-free transitional face of the endoplasmic reticulum (ER) and associated vesicles. The encoded protein plays an important role in the ER-Golgi protein trafficking by promoting the transport of secretory, plasma membrane, and vacuolar proteins from the endoplasmic reticulum to the Golgi complex. Mutation of the SEC23A gene causes Cranio-Lenticular-Sutural Dysplasia (CLSD), a novel autosomal recessive disorder.