[HTML][HTML] The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo
EB Neufeld, M Wastney, S Patel, S Suresh… - Journal of Biological …, 1999 - ASBMB
Niemann-Pick C disease (NP-C) is a neurovisceral lysosomal storage disorder. A variety of
studies have highlighted defective sterol trafficking from lysosomes in NP-C cells. However,
the heterogeneous nature of additional accumulating metabolites suggests that the cellular
lesion may involve a more generalized block in retrograde lysosomal trafficking.
Immunocytochemical studies in fibroblasts reveal that theNPC1 gene product resides in a
novel set of lysosome-associated membrane protein-2 (LAMP2)(+)/mannose 6-phosphate …
studies have highlighted defective sterol trafficking from lysosomes in NP-C cells. However,
the heterogeneous nature of additional accumulating metabolites suggests that the cellular
lesion may involve a more generalized block in retrograde lysosomal trafficking.
Immunocytochemical studies in fibroblasts reveal that theNPC1 gene product resides in a
novel set of lysosome-associated membrane protein-2 (LAMP2)(+)/mannose 6-phosphate …