2019

Zeng R, Sidik H, Robinson KS, Zhong FL, Reversade B, Pouladi MA. Generation of four H1 hESC sublines carrying a hemizygous knock-out/mutant MECP2. Stem Cell Research. 2019. doi:10.1016/j.scr.2019.101533

Ziaei A, Xu X, Dehghani L, Bonnard C, Zellner A, Jin Ng AY, Tohari S, Venkatesh B, Haffner C, Reversade B, Shaygannejad V, Pouladi MA. Novel mutation in HTRA1 in a family with diffuse white matter lesions and inflammatory features. Neurol Genet. 2019. 5(4):e345–10.

Ferrari Bardile C, Garcia-Miralles M, Caron NS, Rayan NA, Langley SR, Harmston N, Rondelli AM, Teo RTY, Waltl S, Anderson LM, Bae HG, Jung S, Williams A, Prabhakar S, Petretto E, Hayden MR, Pouladi MA. Intrinsic mutant HTT-mediated defects in oligodendroglial cells cause myelin deficits and behavioural abnormalities in Huntington disease. PNAS. doi:10.1073/pnas.1818042116

Sidik H, Ang CJ, Pouladi MA. Huntingtin confers fitness but is not embryonically essential in zebrafish development. BioRxiv. doi:10.1101/615591

Utami KH, Skotte N, Colaço AR, Yusof NABM, Sim B, Yeo XY, Bae HG, Radulescu C, Garcia-Miralles M, Chen Q, Chaldaiopoulou G, Liany H, Nama S, Sampath P, Jung S, Mann M, Pouladi MA. Integrative analysis identifies key molecular signatures underlying neurodevelopmental deficits in Fragile X Syndrome. BioRxiv. doi:10.1101/606038

van Kuilenburg ABP*, Tarailo-Graovac M*, Richmond PA*, Drögemöller BI, Pouladi MA, Leen R, Brand-Arzamendi K, Dobritzsch D, Dolzhenko E, Eberle MA, Hayward B, Jones MJ, Karbassi F, Kobor MS, Koster J, Kumari D, Li M, MacIsaac J, McDonald C, Meijer J, Nguyen C, Rajan-Babu IS, Scherer SW, Sim B, Trost B, Tseng LA, Turkenburg M, van Vugt JJFA, Veldink JH, Walia JS, Wang Y, van Weeghel M, Wright GEB, Xu X, Yuen RKC, Zhang J, Ross CJ, Wasserman WW, Geraghty MT, Santra S, Wanders RJA, Wen XY, Waterham HR, Usdin K, van Karnebeek CDM. Glutaminase deficiency caused by short tandem repeat expansion in GLS. New England Journal of Medicine. 2019. 380:1433-41. PMID:30970188.

Teo RTY, Ferrari Bardile C, Tay YL, Yusof NABM, Kreidy CA, Tan LJ, Pouladi MA. Impaired remyelination in a mouse model of Huntington disease. Molecular Neurobiology. 2019. PMID:30937636. [PDF]

Radulescu CI, Garcia-Miralles M, Sidik H, Bardile CF, Yusof NABM, Ung LH, Ho X, Chu W, Layton E, Low D, De Sessions PF, Pettersson S, Ginhoux F, Pouladi MA. Manipulation of microbiota reveals altered callosal myelination and white matter plasticity in a model of Huntington disease. Neurobiology of Disease. 2019. PMID:30802499. [PDF]

Ooi J*, Langley S*, Xu X*, Utami KH*, Sim B, Huang Y, Harmston NP, Tay YL, Ziaei A, Zeng R, Low D, Aminkeng F, Sobota RM, Ginhoux F, Petretto EG, Pouladi MA. Unbiased profiling of isogenic Huntington disease hPSC-derived CNS and peripheral cells reveals strong cell-type-specificity of CAG length effects. Cell Reports. 2019. 26(9): P2494-2508.E7. [PDF]

Garcia-Miralles M, Yusof NABM, Tan JY, Radulescu CI, Sidik H, Tan LJ, Zach N, Hayden MR, Pouladi MA. Laquinimod treatment improves myelination deficits at the transcriptional and ultrastructural levels in the YAC128 mouse model of Huntington disease. Molecular Neurobiology. 2019. PMID:30334188. [PDF]

2018

Chang WT, Puspitasari F, Garcia-Miralles M, Yeowa LY, Tay HC, Koh KB, Tan LJ, Pouladi MA, Chuang KH. Connectomic imaging reveals Huntington-related pathological and pharmaceutical effects in a mouse model. NMR in Biomedicine. 2018. PMID:30260561. [PDF]

Kusko R, Dreymann J, Ross J, Cha Y, Escalante-Chong R, Garcia-Miralles M, Tan LJ, Burczynski ME, Zeskind B, Laifenfeld D, Pouladi MA, Geva M, Grossman I, Hayden MR. Large-scale transcriptomic analysis reveals that pridopidine reverses aberrant gene expression and activates neuroprotective pathways in the YAC128 HD mouse. Molecular Neurodegeneration. 2018. PMID:29783994. 

Chia PH, Zhong FL, Niwa S, Bonnard C, Utami KH, Zeng R, Lee H, Eskin A, Nelson A, Xie W, Al-Tawalbeh S, El-Khateeb M, Shboul M, Pouladi MA, Al-Raqad M, Reversade B. A homozygous loss-of-function CAMK2A mutation causes growth delay, frequent seizures and severe intellectual disabilityeLife. 2018. PMID:29784083.

2017

Garcia-Miralles M, Geva M, Tan JY, Yusof NA, Cha Y, Kusko R, Tan LJ, Xu X, Grossman I, Orbach A, Hayden MR, Pouladi MA. Early pridopidine treatment improves behavioural and transcriptional deficits in Huntington disease mice. JCI Insight. 2017. PMID:29212949. [PDF]

Xu X, Utami KH, Radulescu CI, Pouladi MA. Obtaining multi-electrode array recordings from human induced pluripotent stem cell-derived neurons. Bio-protocol. 2017. doi:10.21769/BioProtoc.2609. [PDF]

Chen MZ, Moily NS, Bridgford J, Wood RJ, Radwan M, Bridgford J, Smith TA, Song Z, Tang BZ, Tilley L, Xu X, Reid GE, Pouladi MA, Hong Y, Hatters DM. A thiol probe for measuring unfolded protein load and proteostasis in cells. Nature Communications. 2017. PMID:28883394.

Takata K, Kozaki T, Lee CZW, Thion MS, Otsuka M, Lim S, Utami KH, Fidan K, Park DS, Malleret B, Chakarov S, See P, Low D, Low G, Garcia-Miralles M, Zeng R, Zhang J, et al. Garel S, Pouladi MA, Ginhoux F. Induced-Pluripotent-Stem-Cell-Derived Primitive Macrophages Provide a Platform for Modeling Tissue-Resident Macrophage Differentiation and Function. Immunity. 2017. PMID:28723550.

Xu X, Tay YL, Sim B, Yoon SI, Huang Y, Ooi J, Utami KH, Radulescu C, Low D, Loh M, Ginhoux F, Augustine GJ, Pouladi MA. Reversal of Phenotypic Abnormalities by CRISPR/Cas9-Mediated Gene Correction in Huntington Disease Patient-Derived Induced Pluripotent Stem Cells. Stem Cell Reports. 2017. PMID:28238795. [PDF]

Southwell AL, Skotte NH, Villanueva EB, Østergaard ME, Gu X, Kordasiewicz HB, Kay C, Cheung D, Xie Y, Waltl S, Dal Cengio L, Doty CN, Petoukhov E, Findlay-Black H, Iworima D, Slama R, Ooi J, Pouladi MA, Yang XW, Swayze EE, Seth PP, Hayden MR. A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles. Human Molecular Genetics. 2017. PMID:28104789.

Julien SG, Kim SY, Brunmeir R, Sinnakannu JR, Ge X, Li H, Ma W, Yaligar J, Prakash B, Velan SS, Röder PV, Zhang Q, Sim CK, Wu J, Garcia-Miralles M, Pouladi MA, Xie W, McFarlane C, Han W, Xu F. Narciclasine attenuates diet-induced obesity by promoting oxidative metabolism in skeletal muscle. PLoS Biology. 2017. PMID:28207742.

2016

Garcia-Miralles M, Hong X, Tan LJ, Caron N, Huang Y, Lin R, Franciosi S, Hayardeny L, Hayden MR, Chuang KH, Pouladi MALaquinimod rescues striatal and white matter pathology and results in modest improvements in motor and depressive phenotypes in the YAC128 model of Huntington diseaseSci Rep.  2016. PMID:27528441. [PDF]

Itahana Y, Zhang J, Göke J, Vardy LA, Han R, Iwamoto K, Cukuroglu E, Robson P, Pouladi MA, Colman A, Itahana K. Histone modifications and p53 binding poise the p21 promoter for activation in human embryonic stem cells. Sci Rep.  2016. PMID:27346849 | PMCID:PMC4921813

Maillet A, Tan K, Chai X, Sadananda SN, Mehta A, Ooi J, Hayden MR, Pouladi MA, Ghosh S, Shim W, Brunham LR. Modeling Doxorubicin-Induced Cardiotoxicity in Human Pluripotent Stem Cell Derived-Cardiomyocytes. Sci Rep.  2016. PMID:27142468 | PMCID:PMC4855185

Teo RT, Hong X, Yu-Taeger L, Huang Y, Tan LJ, Xie Y, To XV, Guo L, Rajendran R, Novati A, Calaminus C, Riess O, Hayden MR, Nguyen HP, Chuang KH, Pouladi MA. Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease. Hum Mol Genet.  2016. PMID:27126634 [PDF]

Riechers SP, Butland S, Deng Y, Skotte N, Ehrnhoefer DE, Russ J, Laine J, Laroche M, Pouladi MA, Wanker EE, Hayden MR, Graham RK. Interactome network analysis identifies multiple caspase-6 interactors involved in the pathogenesis of HD. Hum Mol Genet.  2016. PMID:26908611

Garcia-Miralles M, Ooi J, Ferrari Bardile C, Tan LJ, George M, Drum CL, Lin RY, Hayden MR, Pouladi MA. Treatment with the MAO-A inhibitor clorgyline elevates monoamine neurotransmitter levels and improves affective phenotypes in a mouse model of Huntington disease. Exp Neurol.  2016. PMID:26825854 [PDF]

2015

Southwell AL, Franciosi S, Villanueva EB, Xie Y, Winter LA, Veeraraghavan J, Jonason A, Felczak B, Zhang W, Kovalik V, Waltl S, Hall G, Pouladi MA, Smith ES, Bowers WJ, Zauderer M, Hayden MR. Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease. Neurobiol Dis.  2015. PMID:25662335

Wong BK, Ehrnhoefer DE, Graham RK, Martin DD, Ladha S, Uribe V, Stanek LM, Franciosi S, Qiu X, Deng Y, Kovalik V, Zhang W, Pouladi MA, Shihabuddin LS, Hayden MR. Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice. Neurobiol Dis.  2015. PMID:25583186

Ooi J, Hayden MR, Pouladi MA. Inhibition of Excessive Monoamine Oxidase A/B Activity Protects Against Stress-induced Neuronal Death in Huntington Disease. Mol Neurobiol.  2015. PMID:25398695 | PMCID:PMC4586002 [PDF]

2014

Philips T, Rothstein JD, Pouladi MA. Preclinical models: needed in translation? A Pro/Con debate. Mov Disord.  2014. PMID:25216370 [PDF]

2013

Pouladi MA, Morton AJ, Hayden MR. Choosing an animal model for the study of Huntington's disease. Nat Rev Neurosci.  2013. PMID:24052178

Marco S, Giralt A, Petrovic MM, Pouladi MA, Martínez-Turrillas R, Martínez-Hernández J, Kaltenbach LS, Torres-Peraza J, Graham RK, Watanabe M, Luján R, Nakanishi N, Lipton SA, Lo DC, Hayden MR, Alberch J, Wesseling JF, Pérez-Otaño I. Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models. Nat Med.  2013. PMID:23852340 | PMCID:PMC3936794

Southwell AL, Warby SC, Carroll JB, Doty CN, Skotte NH, Zhang W, Villanueva EB, Kovalik V, Xie Y, Pouladi MA, Collins JA, Yang XW, Franciosi S, Hayden MR. A fully humanized transgenic mouse model of Huntington disease. Hum Mol Genet.  2013. PMID:23001568 | PMCID:PMC3606012

2012

Huang YH, Pouladi MA. Heritability of pain sensitivity and opioid analgesia. Clin Genet.  2012. PMID:22880893

Teo RT, Pouladi MA. PS: pain and sodium channels. Clin Genet.  2012. PMID:22880841

Pouladi MA. Painful gains: missense mutations in SCN9A and idiopathic small nerve fibre neuropathy. Clin Genet.  2012. PMID:22880747

Pouladi MA, Brillaud E, Xie Y, Conforti P, Graham RK, Ehrnhoefer DE, Franciosi S, Zhang W, Poucheret P, Compte E, Maurel JC, Zuccato C, Cattaneo E, Néri C, Hayden MR. NP03, a novel low-dose lithium formulation, is neuroprotective in the YAC128 mouse model of Huntington disease. Neurobiol Dis.  2012. PMID:22796360

Pouladi MA, Hayden MR. Polyglutamine diseases and the risk of cancer. Lancet Oncol.  2012. PMID:22652230

Pouladi MA, Stanek LM, Xie Y, Franciosi S, Southwell AL, Deng Y, Butland S, Zhang W, Cheng SH, Shihabuddin LS, Hayden MR. Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice. Hum Mol Genet.  2012. PMID:22328089

Uribe V, Wong BK, Graham RK, Cusack CL, Skotte NH, Pouladi MA, Xie Y, Feinberg K, Ou Y, Ouyang Y, Deng Y, Franciosi S, Bissada N, Spreeuw A, Zhang W, Ehrnhoefer DE, Vaid K, Miller FD, Deshmukh M, Howland D, Hayden MR. Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice. Hum Mol Genet.  2012. PMID:22262731 | PMCID:PMC3315204

Graham RK, Deng Y, Pouladi MA, Vaid K, Ehrnhoefer D, Southwell AL, Bissada N, Franciosi S, Hayden MR. Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo. J Huntingtons Dis.  2012. PMID:25063333

2011

Pouladi M. Fragile X syndrome therapy: to respond or not to respond may be a matter of methylation. Clin Genet.  2011. PMID:21388370

Song W, Chen J, Petrilli A, Liot G, Klinglmayr E, Zhou Y, Poquiz P, Tjong J, Pouladi MA, Hayden MR, Masliah E, Ellisman M, Rouiller I, Schwarzenbacher R, Bossy B, Perkins G, Bossy-Wetzel E. Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med.  2011. PMID:21336284 | PMCID:PMC3051025

Graham RK, Deng Y, Carroll J, Vaid K, Cowan C, Pouladi MA, Metzler M, Bissada N, Wang L, Faull RL, Gray M, Yang XW, Raymond LA, Hayden MR. Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. J Neurosci.  2010. PMID:21068307 | PMCID:PMC3074336

Simpson JM, Gil-Mohapel J, Pouladi MA, Ghilan M, Xie Y, Hayden MR, Christie BR. Altered adult hippocampal neurogenesis in the YAC128 transgenic mouse model of Huntington disease. Neurobiol Dis.  2011. PMID:20875859

2010

Valenza M, Leoni V, Karasinska JM, Petricca L, Fan J, Carroll J, Pouladi MA, Fossale E, Nguyen HP, Riess O, MacDonald M, Wellington C, DiDonato S, Hayden M, Cattaneo E. Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes. J Neurosci.  2010. PMID:20702713 | PMCID:PMC3842469

Milnerwood AJ, Gladding CM, Pouladi MA, Kaufman AM, Hines RM, Boyd JD, Ko RW, Vasuta OC, Graham RK, Hayden MR, Murphy TH, Raymond LA. Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron.  2010. PMID:20152125

Pouladi MA, Xie Y, Skotte NH, Ehrnhoefer DE, Graham RK, Kim JE, Bissada N, Yang XW, Paganetti P, Friedlander RM, Leavitt BR, Hayden MR. Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum Mol Genet.  2010. PMID:20097678 | PMCID:PMC2846162

Pouladi MA. Taking a SUMO off a TRP for bad conduct. Clin Genet.  2010. PMID:20095982

Becanovic K, Pouladi MA, Lim RS, Kuhn A, Pavlidis P, Luthi-Carter R, Hayden MR, Leavitt BR. Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis. Hum Mol Genet.  2010. PMID:20089533 | PMCID:PMC2846159

2009

Okamoto S*, Pouladi MA*, Talantova M*, Yao D, Xia P, Ehrnhoefer DE, Zaidi R, Clemente A, Kaul M, Graham RK, Zhang D, Vincent Chen HS, Tong G, Hayden MR, Lipton SA. Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat Med.  2009. PMID:19915593 | PMCID:PMC2789858 (* co-first)

Ehrnhoefer DE, Butland SL, Pouladi MA, Hayden MR. Mouse models of Huntington disease: variations on a theme. Dis Model Mech.  2009. PMID:19259385 | PMCID:PMC2650190

Graham RK, Pouladi MA, Joshi P, Lu G, Deng Y, Wu NP, Figueroa BE, Metzler M, André VM, Slow EJ, Raymond L, Friedlander R, Levine MS, Leavitt BR, Hayden MR. Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease. J Neurosci.  2009. PMID:19228972 | PMCID:PMC2729178

Pouladi MA, Graham RK, Karasinska JM, Xie Y, Santos RD, Petersén A, Hayden MR. Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin. Brain.  2009. PMID:19224899

MacDonald ML, van Eck M, Hildebrand RB, Wong BW, Bissada N, Ruddle P, Kontush A, Hussein H, Pouladi MA, Chapman MJ, Fievet C, van Berkel TJ, Staels B, McManus BM, Hayden MR. Despite antiatherogenic metabolic characteristics, SCD1-deficient mice have increased inflammation and atherosclerosis. Arterioscler Thromb Vasc Biol.  2009. PMID:19095997 | PMCID:PMC2873333

2008

Björkqvist M, Wild EJ, Thiele J, Silvestroni A, Andre R, Lahiri N, Raibon E, Lee RV, Benn CL, Soulet D, Magnusson A, Woodman B, Landles C, Pouladi MA, Hayden MR, Khalili-Shirazi A, Lowdell MW, Brundin P, Bates GP, Leavitt BR, Möller T, Tabrizi SJ. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med.  2008. PMID:18625748 | PMCID:PMC2525598

2007

Kuhn A, Goldstein DR, Hodges A, Strand AD, Sengstag T, Kooperberg C, Becanovic K, Pouladi MA, Sathasivam K, Cha JH, Hannan AJ, Hayden MR, Leavitt BR, Dunnett SB, Ferrante RJ, Albin R, Shelbourne P, Delorenzi M, Augood SJ, Faull RL, Olson JM, Bates GP, et al. Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet.  2007. PMID:17519223

2005 and earlier

Robbins CS*, Pouladi MA*, Fattouh R, Dawe DE, Vujicic N, Richards CD, Jordana M, Inman MD, Stampfli MR. Mainstream cigarette smoke exposure attenuates airway immune inflammatory responses to surrogate and common environmental allergens in mice, despite evidence of increased systemic sensitization. J Immunol.  2005. PMID:16116169 (* co-first)

Fattouh R, Pouladi MA, Alvarez D, Johnson JR, Walker TD, Goncharova S, Inman MD, Jordana M. House dust mite facilitates ovalbumin-specific allergic sensitization and airway inflammation. Am J Respir Crit Care Med.  2005. PMID:15879422

Drannik AG, Pouladi MA, Robbins CS, Goncharova SI, Kianpour S, Stämpfli MR. Impact of cigarette smoke on clearance and inflammation after Pseudomonas aeruginosa infection. Am J Respir Crit Care Med.  2004. PMID:15317669

Swirski FK, Gajewska BU, Robbins CS, D'Sa A, Johnson JR, Pouladi MA, Inman MD, Stämpfli MR. Concomitant airway expression of granulocyte-macrophage colony-stimulating factor and decorin, a natural inhibitor of transforming growth factor-beta, breaks established inhalation tolerance. Eur J Immunol.  2004. PMID:15307170

Robbins CS, Dawe DE, Goncharova SI, Pouladi MA, Drannik AG, Swirski FK, Cox G, Stämpfli MR. Cigarette smoke decreases pulmonary dendritic cells and impacts antiviral immune responsiveness. Am J Respir Cell Mol Biol.  2004. PMID:12920055

Pouladi MA, Robbins CS, Swirski FK, Cundall M, McKenzie AN, Jordana M, Shapiro SD, Stämpfli MR. Interleukin-13-dependent expression of matrix metalloproteinase-12 is required for the development of airway eosinophilia in mice. Am J Respir Cell Mol Biol.  2004. PMID:12842850

Coppolino MG, Dierckman R, Loijens J, Collins RF, Pouladi M, Jongstra-Bilen J, Schreiber AD, Trimble WS, Anderson R, Grinstein S. Inhibition of phosphatidylinositol-4-phosphate 5-kinase Ialpha impairs localized actin remodeling and suppresses phagocytosis. J Biol Chem. 2002. PMID:12223494