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null Wei-Hsiang Huang, PhD

Junior Scientist, RI-MUHC, Montreal General Hospital site

Brain Repair and Integrative Neuroscience (BRaIN) Program

Centre for Translational Biology

Assistant Professor, Department of Neurology and Neurosurgery, Faculty of Medicine and Health Sciences, McGill University

 

Keywords

autism spectrum disorders • neurodevelopment • epilepsy • ADHD • genetic mouse models • neural stem cells • human iPSC-derived 2D and 3D neuronal models

Research Focus

My research focuses on understanding how gene dosage imbalance causes human neurodevelopmental disorders including autism, epilepsy, ADHD, and other neuropsychiatric conditions. My lab uses innovative human stem cell and mouse models to dissect how copy number variants affect neural functions on molecular, cellular, circuit, and behavioural levels. We are currently interested in Smith-Magenis syndrome, caused by loss of a transcription factor, RAI1.

Selected Publications

Click on Pubmed to see my current publications list

  • Chang YT, Lee YJ, Haque M, Chang HC, Javed S, Lin YC, Cho Y, Abramovitz J, Chin G, Khamis A, Raja R, Murai KK, Huang WH. Comparative analyses of the Smith-Magenis syndrome protein RAI1 in mice and common marmoset monkeys. J Comp Neurol. 2024 Jan;532(1):e25589. doi: 10.1002/cne.25589. PMID: 38289192.

  • Javed S, Chang YT, Cho Y, Lee YJ, Chang HC, Haque M, Lin YC, Huang WH. Smith-Magenis syndrome protein RAI1 regulates body weight homeostasis through hypothalamic BDNF-producing neurons and neurotrophin downstream signalling. Elife. 2023 Nov 13;12:RP90333. doi: 10.7554/eLife.90333. PMID: 37956053.

  • Chang HC, Lee YJ, Javed S, Haque M, Chang YT, Lin YC, Oram C, Huang WH. rAAV-CRISPRa therapy corrects Rai1 haploinsufficiency and rescues selective disease features in Smith-Magenis syndrome mice. J Biol Chem. 2023 Jan;299(1):102728. doi: 10.1016/j.jbc.2022.102728. Epub 2022 Nov 19. PMID: 36410433.

  • Chang YT, Kowalczyk M, Fogerson PM, Lee YJ, Haque M, Adams EL, Wang DC, DeNardo LA, Tessier-Lavigne M, Huguenard JR, Luo L, Huang WH. Loss of Rai1 enhances hippocampal excitability and epileptogenesis in mouse models of Smith-Magenis syndrome. Proc Natl Acad Sci U S A. 2022 Oct 25;119(43):e2210122119. doi: 10.1073/pnas.2210122119. Epub 2022 Oct 18. Erratum in: Proc Natl Acad Sci U S A. 2022 Dec 27;119(52):e2219265119. doi: 10.1073/pnas.2219265119. PMID: 36256819.

  • Javed S, Lee YJ, Xu J, Huang WH. Temporal dissection of Rai1 function reveals brain-derived neurotrophic factor as a potential therapeutic target for Smith-Magenis syndrome. Hum Mol Genet. 2021 Dec 27;31(2):275-288. doi: 10.1093/hmg/ddab245. PMID: 34463714.