A glycoprotein growth factor (epithelin precursor, proepithelin, acrogranin, 593 aa) that has seven similar cysteine-rich domains; these are cleaved from progranulin and some were originally isolated as granulins (granulins A–D). Progranulin is expressed in many epithelial cells, macrophages, and monocyte-derived dendritic cells and is involved in multiple physiological and pathological processes. The granulins are also active in various processes. Mutations in the progranulin gene have been shown to cause familial frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTDL-U). Progranulin mediates proteolytic cleavage of TDP-43 to generate ~35-kDa and ~25-kDa species.
Subjects: Medicine and Health.