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Polycystins: what polycystic kidney disease tells us about sperm
Author(s) -
Kierszenbaum Abraham L.
Publication year - 2004
Publication title -
molecular reproduction and development
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.745
H-Index - 105
eISSN - 1098-2795
pISSN - 1040-452X
DOI - 10.1002/mrd.20042
Subject(s) - biology , polycystic kidney disease , cilium , pkd1 , microbiology and biotechnology , sperm , cystic kidney disease , medicine , endocrinology , kidney , genetics
Experimental evidence indicates that the membrane‐associated proteins polycystin‐1 and polycystin‐2 operate as a receptor‐calcium channel complex that regulates signaling pathways essential for modulation of renal tubulogenesis. Polycystic kidney disease is characterized by defective renal tubular structure and results from mutations in either PKD1 or PKD2 genes. Recent data suggest that polycystin‐1 and polycystin‐2 might localize to primary cilium in principal cells of renal collecting tubules and are thought to act as mechanosensors of fluid flow and contents. Ciliary bending by fluid flow or mechanical stimulation induce Ca 2+ release from intracellular stores, presumably to modulate ion influx in response to tubular fluid flow. Polycystins are also emerging as playing a significant role in sperm development and function. Drosophila polycystin‐2 is associated with the head and tail of mature sperm. Targeted disruption of the PKD2 homolog results in nearly complete male sterility without disrupting spermatogenesis. Mutant sperm are motile but are unable to reach the female storage organs (seminal receptacles and spermathecae). The sea urchin polycystin‐1‐equivalent suPC2 colocalizes with the polycystin‐1 homolog REJ3 to the plasma membrane over the acrosomal vesicle. This localization site suggests that the suPC2‐REJ3 complex may function as a cation channel mediating acrosome reaction when sperm contact the jelly layer surrounding the egg at fertilization. Future studies leading to the identification of specific ligands for polycystins, including the signaling pathways, might define the puzzling relationship between renal tubular morphogenesis and sperm development and function. Mol. Reprod. Dev. 67: 385–388, 2004. © 2004 Wiley‐Liss, Inc.