INVESTIGADORES
CANTIELLO Horacio Fabio
artículos
Título:
Submembraneous microtubule cytoskeleton: interaction of TRPP2 with the cell cytoskeleton
Autor/es:
XING-ZHEN CHEN, QIANG LI, YULIANG WU, GENQING LIANG, CARLOS J. LARA,HORACIO F. CANTIELLO
Revista:
FEBS JOURNAL
Referencias:
Año: 2008 vol. 275 p. 4675 - 4683
ISSN:
1742-464X
Resumen:
TRPP2, also called polycystin-2, the gene product of PKD2, is a membrane
protein defective in 1015% of cases of autosomal dominant polycystic
kidney disease. Mutations in PKD2 are also associated with extrarenal disorders,
such as hepatic cystogenesis and cardiovascular abnormalities.
TRPP2 is a Ca-permeable nonselective cation channel present in the endoplasmic
reticulum and plasma membrane, as well as in cilia of renal epithelial
and embryonic nodal cells, in which it likely forms part of a flow
sensor. Recent studies have identified a number of TRPP2-interacting proteins,
of which many are cytoskeletal components. Work from our and
other laboratories indicates that cytoskeletal partner proteins seem to play
important, albeit highly complex, roles in the regulation of TRPP2 expression,
localization and channel function. This minireview covers current
knowledge about cytoskeletal interactions with TRPP2, and suggests that
mutations in proteins of the TRPP2-cytoskeleton complex may be implicated
in the pathogenesis of autosomal dominant polycystic kidney disease.
protein defective in 1015% of cases of autosomal dominant polycystic
kidney disease. Mutations in PKD2 are also associated with extrarenal disorders,
such as hepatic cystogenesis and cardiovascular abnormalities.
TRPP2 is a Ca-permeable nonselective cation channel present in the endoplasmic
reticulum and plasma membrane, as well as in cilia of renal epithelial
and embryonic nodal cells, in which it likely forms part of a flow
sensor. Recent studies have identified a number of TRPP2-interacting proteins,
of which many are cytoskeletal components. Work from our and
other laboratories indicates that cytoskeletal partner proteins seem to play
important, albeit highly complex, roles in the regulation of TRPP2 expression,
localization and channel function. This minireview covers current
knowledge about cytoskeletal interactions with TRPP2, and suggests that
mutations in proteins of the TRPP2-cytoskeleton complex may be implicated
in the pathogenesis of autosomal dominant polycystic kidney disease.
PKD2, is a membrane
protein defective in 1015% of cases of autosomal dominant polycystic
kidney disease. Mutations in PKD2 are also associated with extrarenal disorders,
such as hepatic cystogenesis and cardiovascular abnormalities.
TRPP2 is a Ca-permeable nonselective cation channel present in the endoplasmic
reticulum and plasma membrane, as well as in cilia of renal epithelial
and embryonic nodal cells, in which it likely forms part of a flow
sensor. Recent studies have identified a number of TRPP2-interacting proteins,
of which many are cytoskeletal components. Work from our and
other laboratories indicates that cytoskeletal partner proteins seem to play
important, albeit highly complex, roles in the regulation of TRPP2 expression,
localization and channel function. This minireview covers current
knowledge about cytoskeletal interactions with TRPP2, and suggests that
mutations in proteins of the TRPP2-cytoskeleton complex may be implicated
in the pathogenesis of autosomal dominant polycystic kidney disease.