Last modified: July 31, 2014

Protein trafficking is essential for many cellular processes inside the cell.  For example, newly synthesized transmembrane and luminal/extracellular proteins need to be delivered through secretory pathways to reach their final destination for proper functions.  On the other hand, membrane proteins on the cell surface need to be endocytosed and transported to lysosomes for their degradation.   Many proteins are dedicated in the transport processes, such as COPI coats mediating vesicular transport from the Golgi to the ER, COPII coats mediating transport from the ER to the Golgi, and clathrin coats and AP complexes mediating transport between plasma membrane and the trans-Golgi network.   Understanding how the transport machineries operate is therefore very important.  We have previously identified a negative regulator that mediates COPI transport.  We are expanding on this finding. 

In addition to basic research in transport mechanisms, we are also interested in transport defects in diseases.  Many lysosomal storage disorders have faulty lysosomal enzymes due to improper transport and processing of the enzymes.  Currently, we focus on neuronal-ceroid lipofuscinoses (NCLs), a group of childhood neurodegenerative disorders.  NCL is categorized as a lysosomal storage disorder.  Currently we are working on three proteins, CLN5, CLN6, and CLN8.  Mutations of CLN5, CLN6, or CLN8 can lead to variant late infantile NCLs.