Gaucher disease: forging a new path to the lysosome.
Most lysosomal proteins are tagged with a carbohydrate modification that allows their recognition and transport to the lysosome. In this issue, Reczek et al. (2007) identify a new pathway for protein sorting to the lysosome. They show that beta-glucocerebrosidase-the lysosomal enzyme defective in patients with Gaucher disease-is delivered to the lysosome through its interaction with the transmembrane protein LIMP-2.
Griffiths
Gillian M
GM
Cambridge Institute for Medical Research, Wellcome/MRC Building, Box 139, Addenbrooke's Hospital, Hills Road, Cambridge CB2 0XY. gg305@cam.ac.uk
D005776:Gaucher Disease / Q000378:metabolism*; D005962:Glucosylceramidase / Q000378:metabolism; D006801:Humans; D051907:Lysosomal Membrane Proteins / Q000378:metabolism; D008247:Lysosomes / Q000378:metabolism*; D020033:Protein Isoforms / Q000378:metabolism; D021381:Protein Transport
D051907:Lysosomal Membrane Proteins; D020033:Protein Isoforms; D005962:Glucosylceramidase