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The TRPML3 Channel: From Gene to Function

  • ️Fri Dec 24 2010

Abstract

TRPML3 is a transient receptor potential (TRP) channel that is encoded by the mucolipin 3 gene (MCOLN3), a member of the small mucolipin gene family. Mcoln3 shows a broad expression pattern in embryonic and adult tissues that includes differentiated cells of skin and inner ear. Dominant mutant alleles of murine Mcoln3 cause embryonic lethality, pigmentation defects and deafness. The TRPML3 protein features a six-transmembrane topology and functions as a Ca2+ permeable inward rectifying cation channel that is open at sub-physiological pH and closes as the extracytosolic pH becomes more acidic. TRPML3 localizes to the plasmamembrane and to early- and late-endosomes as well as lysosomes. Recent advances suggest that TRPML3 may regulate the acidification of early endosomes, hence playing a critical role in the endocytic pathway.

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Acknowledgments

I thank Rosa Puertollano and Jamie García-Añoveros for discussions and comments on the manuscript. Supported by the Intramural Research Program at the National Institute on Deafness and Other Communication Disorders.

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Authors and Affiliations

  1. Section on Neurogenetics, Laboratory of Molecular Biology, National Institute on Deafness and Other Communication Disorders, Rockville, MD, 20850, USA

    Konrad Noben-Trauth

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  1. Konrad Noben-Trauth

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Correspondence to Konrad Noben-Trauth .

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Editors and Affiliations

  1. Dept. Clinical Research & Education, Södersjukhuset, Karolinska Institutet, Stockholm, 118 83, Sweden

    Md. Shahidul Islam

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Noben-Trauth, K. (2011). The TRPML3 Channel: From Gene to Function. In: Islam, M. (eds) Transient Receptor Potential Channels. Advances in Experimental Medicine and Biology, vol 704. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-0265-3_13

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