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Nam Chul Kim
Researcher at University of Minnesota
Publications - 16
Citations - 2823
Nam Chul Kim is an academic researcher from University of Minnesota. The author has contributed to research in topics: Multisystem proteinopathy & PINK1. The author has an hindex of 10, co-authored 15 publications receiving 2363 citations. Previous affiliations of Nam Chul Kim include University of Alabama at Birmingham & St. Jude Children's Research Hospital.
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Journal ArticleDOI
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS
Hong Joo Kim,Nam Chul Kim,Yong Dong Wang,Emily A. Scarborough,Jennifer Moore,Zamia Diaz,Kyle S. MacLea,Brian D. Freibaum,Songqing Li,Amandine Molliex,Anderson P. Kanagaraj,Robert A. Carter,Kevin B. Boylan,Aleksandra Wojtas,Rosa Rademakers,Jack L. Pinkus,Steven A. Greenberg,John Q. Trojanowski,Bryan J. Traynor,Bradley N. Smith,Simon Topp,Athina Soragia Gkazi,Jack W. Miller,Christopher Shaw,Michael Kottlors,Janbernd Kirschner,Alan Pestronk,Yun Li,Alice Flynn Ford,Aaron D. Gitler,Michael Benatar,Oliver D. King,Virginia Kimonis,Eric D. Ross,Conrad C. Weihl,James Shorter,J. Paul Taylor +36 more
TL;DR: Dysregulated polymerization caused by a potent mutant steric zipper motif in a PrLD can initiate degenerative disease and related proteins with PrLDs should be considered candidates for initiating and perhaps propagating proteinopathies of muscle, brain, motor neuron and bone.
Journal ArticleDOI
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
Brian D. Freibaum,Yubing Lu,Rodrigo Lopez-Gonzalez,Nam Chul Kim,Sandra Almeida,Kyung-Ha Lee,Nisha M. Badders,Marc Valentine,Bruce L. Miller,Philip C. Wong,Leonard Petrucelli,Hong Joo Kim,Fen-Biao Gao,J. Paul Taylor +13 more
TL;DR: It is shown that a primary consequence of G4C2 repeat expansion is the compromise of nucleocytoplasmic transport through the nuclear pore, revealing a novel mechanism of neurodegeneration.
Journal ArticleDOI
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
Kyung-Ha Lee,Peipei Zhang,Hong Joo Kim,Diana M. Mitrea,Mohona Sarkar,Brian D. Freibaum,Jaclyn A Cika,Maura Coughlin,James Messing,Amandine Molliex,Brian A. Maxwell,Nam Chul Kim,Jamshid Temirov,Jennifer Moore,Regina-Maria Kolaitis,Timothy I. Shaw,Bing Bai,Junmin Peng,Richard W. Kriwacki,Richard W. Kriwacki,J. Paul Taylor +20 more
TL;DR: It is found that arginine-containing DPRs, polyGly-Arg (GR) and polyPro- Arg (PR), interact with RNA-binding proteins and proteins with low complexity sequence domains (LCDs) that often mediate the assembly of membrane-less organelles.
Journal ArticleDOI
VCP Is Essential for Mitochondrial Quality Control by PINK1/Parkin and this Function Is Impaired by VCP Mutations
Nam Chul Kim,Emilie Tresse,Regina-Maria Kolaitis,Amandine Molliex,Ruth E. Thomas,Nael H. Alami,Bo Wang,Aashish Joshi,Rebecca B. Smith,Gillian P. Ritson,Brett J. Winborn,Jennifer Moore,Joo-Yong Lee,Tso-Pang Yao,Leo J. Pallanck,Mondira Kundu,J. Paul Taylor +16 more
TL;DR: A Drosophila model of VCP mutation-dependent degeneration is developed that is reminiscent of PINK1 and parkin mutants, including a pronounced mitochondrial defect, and it is shown that VCP recruitment to damaged mitochondria requires Parkin-mediated ubiquitination of mitochondrial targets.
Journal ArticleDOI
Relay of retrograde synaptogenic signals through axonal transport of BMP receptors
TL;DR: Data support a model in which complexes of activated BMP receptors are actively transported along the axon towards the cell body to relay the synaptogenic signal, and that phosphorylated Mad at the synaptic terminal and cell body represent two distinct molecular populations.