Display Settings:


Send to:

Choose Destination
See comment in PubMed Commons below
Commun Integr Biol. 2012 Mar 1;5(2):111-3. doi: 10.4161/cib.20373.

Cross-talk between TRPML1 channel, lipids and lysosomal storage diseases.

Author information

  • Hotchkiss Brain Institute; Department of Physiology and Pharmacology; University of Calgary; Calgary, AB Canada.


Described by the Belgian cytologist Christian De Duve in 1949,(1) lysosomes (from the Greek "digestive bodies") are ubiquitous specialized intracellular organelles that ensure the degradation/recycling of macromolecules (proteins, lipids, membranes) through the activity of specific enzymes (i.e., acid hydrolases). They receive their substrates through different internalization pathways (i.e., endocytosis, phagocytosis and autophagy) and are involved in a wide range of physiological functions from cell death and signaling to cholesterol homeostasis and plasma membrane repair.(2) In Mammals, 50 soluble lysosomal hydrolases have been described, each targeting specific substrates. They are confined in the lumen of the lysosome and require an optimum pH (i.e., pH 4.5) to work. This acidic pH compared with the slightly alkaline pH of the cytosol (i.e., ~pH 7.2) is maintained by the activity of integral lysosomal membrane proteins (LMPs, that represent the second class of lysosomal proteins), including the V-type proton (H(+))-ATPase(3) and the chloride ion channel CLC7(4) that pumps protons from the cytosol across the lysosomal membrane.


Niemann-Pick disease; TRP channel; TRPML1 channel; calcium; ion channel; lipid storage disorder; lysosome; mucolipin; sphingomyelin; trafficking

Free PMC Article

Images from this publication.See all images (1)Free text

PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for PubMed Central
    Loading ...
    Write to the Help Desk