H
Han Xiang Deng
Researcher at Northwestern University
Publications - 109
Citations - 13064
Han Xiang Deng is an academic researcher from Northwestern University. The author has contributed to research in topics: Amyotrophic lateral sclerosis & SOD1. The author has an hindex of 41, co-authored 106 publications receiving 12178 citations. Previous affiliations of Han Xiang Deng include Fudan University & University of Chicago.
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Journal ArticleDOI
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation.
Mark E. Gurney,Haifeng Pu,Arlene Y. Chiu,Mauro C. Dal Canto,Cynthia Y. Polchow,Denise D. Alexander,Jan Caliendo,Afif Hentati,Young W. Kwon,Han Xiang Deng,W. Chen,Ping Zhai,Robert L. Sufit,Teepu Siddique +13 more
TL;DR: In this article, the authors found that mutations of human Cu,Zn superoxide dismutase (SOD) contribute to the pathogenesis of familial amyotrophic lateral sclerosis (ALS).
Journal ArticleDOI
Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase
Han Xiang Deng,Afif Hentati,John A. Tainer,Zafar Iqbal,Annarueber Cayabyab,Wu Yen Hung,Elizabeth D. Getzoff,Ping Hu,Brian Herzfeldt,Raymond P. Roos,Carolyn Warner,Gang Deng,Edwin Soriano,Celestine Smyth,Hans E. Parge,Aftab Ahmed,Allen D. Roses,Robert A. Hallewell,Margaret A. Pericak-Vance,Teepu Siddique +19 more
TL;DR: In this article, single-site mutants in the Cu,Zn superoxide dismutase (SOD) gene occur in patients with the fatal neurodegenerative disorder familial amyotrophic lateral sclerosis.
Journal ArticleDOI
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
Han Xiang Deng,Wenjie Chen,Seong-Tshool Hong,Seong-Tshool Hong,Kym M. Boycott,George Gorrie,George Gorrie,Nailah Siddique,Yi Yang,Faisal Fecto,Yong-Yong Shi,Hong Zhai,Hujun Jiang,Hujun Jiang,Makito Hirano,Makito Hirano,Evadnie Rampersaud,Gerard H. Jansen,Sandra Donkervoort,Eileen H. Bigio,Benjamin Rix Brooks,Kaouther Ajroud,Robert L. Sufit,Jonathan L. Haines,Enrico Mugnaini,Margaret A. Pericak-Vance,Teepu Siddique +26 more
TL;DR: Findings link abnormalities in ubiquilin 2 to defects in the protein degradation pathway, abnormal protein aggregation and neurodegeneration, indicating a common pathogenic mechanism that can be exploited for therapeutic intervention.
Journal ArticleDOI
The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis.
Yi Yang,Afif Hentati,Han Xiang Deng,Omar Dabbagh,Toru Sasaki,Makito Hirano,Wu Yen Hung,Karim Ouahchi,Jianhua Yan,Anser C. Azim,N. Cole,Generoso G. Gascon,Ayesha Yagmour,Mongi Ben-Hamida,Margaret A. Pericak-Vance,Fayçal Hentati,Teepu Siddique +16 more
TL;DR: The predicted sequence of the protein (alsin) may indicate a mechanism for motor-neuron degeneration, as it may include several cell-signaling motifs with known functions, including three associated with guanine-nucleotide exchange factors for GTPases (GEFs).
Journal ArticleDOI
SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis.
Faisal Fecto,Jianhua Yan,S. Pavan Vemula,Erdong Liu,Yi Yang,Wenjie Chen,Jian Guo Zheng,Yong Shi,Nailah Siddique,Hasan Arrat,Sandra Donkervoort,Senda Ajroud-Driss,Robert L. Sufit,Scott Heller,Han Xiang Deng,Teepu Siddique +15 more
TL;DR: The findings provide evidence of a direct genetic role for p62 in ALS pathogenesis and suggest that regulation of protein degradation pathways may represent an important therapeutic target in motor neuron degeneration.