My favorite molecule
The granulin gene family: from cancer to dementia
Article first published online: 30 SEP 2009
Copyright © 2009 Wiley Periodicals, Inc.
Volume 31, Issue 11, pages 1245–1254, November 2009
How to Cite
Bateman, A. and Bennett, H. P. J. (2009), The granulin gene family: from cancer to dementia. Bioessays, 31: 1245–1254. doi: 10.1002/bies.200900086
- Issue published online: 21 OCT 2009
- Article first published online: 30 SEP 2009
- frontotemporal lobar dementia;
The growth factor progranulin (PGRN) regulates cell division, survival, and migration. PGRN is an extracellular glycoprotein bearing multiple copies of the cysteine-rich granulin motif. With PGRN family members in plants and slime mold, it represents one of the most ancient of the extracellular regulatory proteins still extant in modern animals. PRGN has multiple biological roles. It contributes to the regulation of early embryogenesis, to adult tissue repair and inflammation. Elevated PGRN levels often occur in cancers, and PGRN immunotherapy inhibits the growth of hepatic cancer xenografts in mice. Recent studies have demonstrated roles for PGRN in neurobiology. An autosomal dominant mutation in GRN, the gene for PGRN, leads to neuronal atrophy in the frontal and temporal lobes, resulting in the disease frontotemporal lobar dementia. In this review we will discuss current knowledge of the multifaceted biology of PGRN.