Abstract
GCM proteins constitute a small transcription factor family with a DNA-binding domain exhibiting a novel fold composed of two subdomains rigidly held together by coordination of one of two structural zinc cations. In all known cases, GCM proteins exert the role of master regulators: the prototypical family member determines gliogenesis in Drosophila melanogaster, whereas mammalian GCM proteins orchestrate divergent aspects of development and physiology in placenta, kidney, thymus, and parathyroid gland. Recent data point to an involvement of GCM proteins in different pathological contexts, such as preeclampsia, hyper- or hypoparathyroidism, and parathyroid gland tumors.