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Am J Physiol Renal Physiol 296: F1245-F1254, 2009. First published January 21, 2009; doi:10.1152/ajprenal.90522.2008
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REVIEW

TRPMLs: in sickness and in health

Rosa Puertollano1 and Kirill Kiselyov2

1Laboratory of Cell Biology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland; and 2Department of Biological Sciences, University of Pittsburgh, Pittsburgh, Pennsylvania

Submitted 2 September 2008 ; accepted in final form 13 January 2009

ABSTRACT

TRPML1, TRPML2 and TRPML3 belong to the mucolipin family of the TRP superfamily of ion channels. The founding member of this family, TRPML1, was cloned during the search for the genetic determinants of the lysosomal storage disease mucolipidosis type IV (MLIV). Mucolipins are predominantly expressed within the endocytic pathway, where they appear to regulate membrane traffic and/or degradation. The physiology of mucolipins raises some of the most interesting questions of modern cell biology. Their traffic and localization is a multistep process involving a system of adaptor proteins, while their ion channel activity possibly exemplifies the rare cases of regulation of endocytic traffic and hydrolysis by ion channels. Finally, dysregulation of mucolipins results in cell death leading to neurodegenerative phenotypes of MLIV and of the varitint-waddler mouse model of familial deafness. The present review discusses current knowledge and questions regarding this novel family of disease-relevant ion channels with a specific focus on mucolipin regulation and their role in membrane traffic and cell death. Since mucolipins are ubiquitously expressed, this review may be useful for a wide audience of basic biologists and clinicians.

mucolipin; endocytosis; ion channel; membrane traffic; lysosome


Address for reprint requests and other correspondence: R. Puertollano, Laboratory of Cell Biology, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892 (e-mail: puertolr{at}mail.nih.gov). or K. Kiselyov, Dept. of Biological Science, University of Pittsburgh, Pittsburgh, PA 15260 (e-mail: kiselyov{at}pitt.edu)




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S. Patel and R. Docampo
In with the TRP Channels: Intracellular Functions for TRPM1 and TRPM2
Sci. Signal., November 3, 2009; 2(95): pe69 - pe69.
[Abstract] [Full Text] [PDF]


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