Department of Pediatrics, School of Medicine
The Kim laboratory studies mechanisms of COPII vesicle assembly and roles of this process in human development.
Intracellular Protein Trafficking and Human Diseases
Secretory proteins and transmembrane proteins are synthesized in the endoplasmic reticulum (ER). The vast majority of these proteins exit the ER via transport vesicles generated by coat protein complex II (COPII). Proper assembly of these COPII vesicles is essential for cell viability and animal development. My laboratory studies how various cargo molecules are packaged into COPII vesicles and what are the consequences of aberrant COPII vesicle assembly in human.
Specialties / Focus
- Cell Biology
- Cell Division and the Cytoskeleton
- Developmental Biology
- Molecular Genetics
- Molecular Medicine
- Organelle and Membrane Biology
- Signal Transduction
- MCB 212 Cell Biology
- BIS 104 Cell Biology
- Intracellular Protein Trafficking
- Kyungho Kim
- David Lu
- Anna Furuta
Honors and Awards
- Health Science Research Award
- Academic Senate Faculty Research Award
- The American Society for Cell Biology
- 2000 Ph.D. Biochemistry University of Connecticut
- 1992 M.S. Molecular Biology Seoul National University
- 1990 B.S. Zoology Seoul National University
Weaver, K.N., M.E.I. Hallek, R.J. Hopkin, K.L. Sund, M. Henrickson, D. del Gaudio, A. Yuksel, G.O. Acar, M.B. Bober, J. Kim and S.A. Boyadjiev. 2014. Keutel syndrome: Report of two nobel MGP mutations and discussion of clinical overlap with arylsulfatase E deficiency and relapsing polychondritis. Am. J. Med. Genet. A. 164:1062-1068.
Kim, S.D., G. Yagnik, M. Cunningham, J. Kim, and S.A. Boyadjiev. 2014. MAPK/ERK signaling pathway analysis in primary osteoblasts from patients with non-syndromic sagittal craniosynostosis. Cleft Palate Craniofac. J. 51:115-119.
Thanabalasuriar, A., J. Kim and S. Gruenheid. 2013. The inhibition of COPII trafficking is important for intestinal epithelial tight junction disruption during enteropathogenic Escherichia coli and Citrobacter rodentium infection. Microbes Infect. 15:738-744.
Justice, C.M. et al. 2012. A genome-wide association study identifies susceptibility loci for non-syndromic sagittal craniosynostosis near BMP2 and within BBS9. Nat. Genet. 44:1360-1364.
Yagnik, G., A. Ghuman, S.D. Kim, C.G. Stevens, V. Kimonis, J. Stoler, P. Sanchez-Lara, J. Bernstein, C. Naydenov, H. Drissi, M.L. Cunningham, J. Kim., and S.A. Boyadjiev. 2012. ALX4 gain-of-function mutations in non-syndromic craniosynostosis. Hum. Mut. 33:1626-1629.
Kim, S.D., J.L. Liu, T. Roscioli, M.F. Buckley, G. Yagnik, S.A. Boyadjiev, and J. Kim. 2012. Leucine-rich repeat, immunoglobulin-like and transmembrane domain 3 (LRIT3) is a modulator of FGFR1. FEBS Lett. 586:1516-1512.
Thanabalasuriar, A., J. Bergeron, A. Gillingham, M. Mimee, J.-L. Thomassin, N. Strynadka, J. Kim, and S. Gruenheid. 2012. Sec24 interaction is essential for localization and virulence-associated function of the bacterial effector protein NleA. Cell. Microbiol. 14:1206-1218.
Fromme, J.C., and J. Kim. 2012. A rapid and quantitative COPII vesicle formation assay using luciferase reporters. Anal. Biochem. 421:482-488.
Kim, S.D., K.B. Pahuja, M. Ravazzola, J. Yoon, S.A. Boyadjiev, S. Hamamoto, R. Schekman, L. Orci, and J. Kim. 2012. The SEC23-SEC31 interface plays a critical role for export of procollagen from the endoplasmic reticulum. J. Biol. Chem. 287:10134-10144.
Zhang, B., C. Zheng, M. Zhu, J. Tao, M. Vasievich, A. Baines, J. Kim, R. Schekman, R.J. Kaufman, and D. Ginsburg. 2011. Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of alpha1-antitrypsin. Blood 118:3384-3391.
Boyadjiev, S.A., S.D. Kim, A. Hata, C. Haldeman-Englert, E.H. Zackai, C. Naydenov, S. Hamamoto, R.W. Schekman, and J. Kim. 2011. Cranio-lenticulo-sutural dysplasia associated with defects in collagen secretion. Clin. Genet. 80:169-176.
Kim, S.D., and J. Kim. 2008. Sequence analyses of presenilin mutations linked to familial Alzheimer’s disease. Cell Stress & Chaperones 13:401-412.
Kim, J., B. Kleizen, R. Choy, G. Thinakaran, S.S. Sisodia, and R. Schekman. 2007. Biogenesis of gamma–secretase early in the secretory pathway. J. Cell Biol. 179:951-963.
Kim, J., A. Thanabalasuriar, T. Chaworth-Musters, J.C. Fromme, E.A. Frey, P.I. Lario, P. Metalnikov, K. Rizg, N.A. Thomas, S.F. Lee. E.L. Hartland, P.R. Hardwidge, T. Pawson, N.C. Strynadka, B.B. Finlay, R. Schekman, and S. Gruenheid. 2007. The bacterial virulence factor NleA inhibits cellular protein secretion by disrupting mammalian COP II function. Cell Host & Microbe 2:160-171.