Centrosomal protein of 126 kDa (or Cep126) is a protein that in humans is encoded by the CEP126 gene (previously KIAA1377). The Cep126 protein has been shown to localize to the centrosome. Furthermore, it is found at pericentriolar satellites and the base of the primary cilium. Depleting Cep126 leads to dispersion of pericentriolar satellites, in turn disrupting microtubule organization at the mitotic spindle.
Clinical relevance Mutations in this gene have been found to cause monomelic amyotrophy.
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