Publications

Sufu- and Spop-mediated downregulation of Hedgehog signaling promotes beta cell differentiation through organ-specific niche signals. Yung T, Poon F, Liang M, Coquenlorge S, McGaugh EC, Hui CC, Wilson MD, Nostro MC, Kim TH. Nature Communications. 2019 Oct 11;10(1):4647.
Activation of Hedgehog Signaling Promotes Development of Mouse and Human Enteric Neural Crest Cells, Based on single-cell Transcriptome Analyses. Lau ST, Li Z, Lai FP, Lui KN, Li P, Munera JO, Pan G, Mahe MM, Hui CC, Wells JM, Ngan ES. Gastroenterology. 2019 Aug 20.

GLI2 modulated by SUFU and SPOP induces intestinal stem cell niche signals in development and tumorigenesis.
Coquenlorge-Gallon S*, Yin WC*, Yung T, Pan J, Zhang X, Mo R, Belik J, Hui CC, Kim TH (* equal contribution). Cell Reports. (2019) Jun 4;27(10):3006-3018.
Thermogenesis-independent metabolic benefits conferred by isocaloric intermittent fasting in ob/ob mice. Kim YH, Lee JH, Yeung JL, Das E, Kim RY, Jiang Y, Moon JH, Jeong H, Thakkar N, Son JE, Trzaskalski N, Hui CC, Doh KO, Mulvihill EE, Kim JR, Kim KH, Sung HK. Science Reports. 2019 Feb 21;9(1):2479.
Dual Regulatory Functions of SUFU and Targetome of GLI2 in SHH Subgroup Medulloblastoma. Yin WC, Satkunendran T, Mo R, Morrissy S, Zhang X, Huang ES, Uusküla-Reimand L, Hou H, Son JE, Liu W, Liu YC, Zhang J, Parker J, Wang X, Farooq H, Selvadurai H, Chen X, Sau-Wai Ngan E, Cheng SY, Dirks PB, Angers S, Wilson MD, Taylor MD, Hui CC. Developmental Cell. 2019 Jan 28;48(2):167-183.
The Iroquois homeobox proteins IRX3 and IRX5 have distinct roles in Wilms tumour development and human nephrogenesis. Holmquist Mengelbier L, Lindell-Munther S, Yasui H, Jansson C, Esfandyari J, Karlsson J, Lau K, Hui CC, Bexell D, Hopyan S, Gisselsson D. Journal of Pathology. 2019 Jan;247(1):86-98.

A Feedforward Mechanism Mediated by Mechanosensitive Ion Channel PIEZO1 and Tissue Mechanics Promotes Glioma Aggression. Chen X, Wanggou S, Bodalia A, Zhu M, Dong W, Fan JJ, Yin WC, Min HK, Hu M, Draghici D, Dou W, Li F, Coutinho FJ, Whetstone H, Kushida MM, Dirks PB, Song Y, Hui CC, Sun Y, Wang LY, Li X, Huang X. Neuron. 2018 Nov 21;100(4):799-815.

Single nucleotide polymorphisms of HAAO and IRX 6 genes as risk factors for hypospadias. Kojima Y, Koguchi T, Mizuno K, Sato Y, Hoshi S, Hata J, Nishio H, Hashimoto D, Matsushita S, Suzuki K, Miyagawa S, Hui CC, Tanikawa C, Murakami Y, Yamada G, Hayashi Y, Matsuda K. Journal of Urology. 2018 Jul 28.

Dynamic expression patterns of Irx3 and Irx5 during germline nest breakdown and primordial follicle formation promote follicle survival in mouse ovaries. Fu A, Oberholtzer SM, Bagheri-Fam S, Rastetter RH, Holdreith C, Caceres VL, John SV, Shaw SA, Krentz KJ, Zhang X, Hui CC, Wilhelm D, Jorgensen JS. PLoS Genetics. 2018 Aug 2;14(8)

Genetic interaction between Gli3 and Ezh2 during limb pattern formation. Deimling SJ, Lau K, Hui CC, Hopyan S. Mechanisms of Development. 2018 Jun;151:30-36.

Intermittent fasting promotes adipose thermogenesis and metabolic homeostasis via VEGF-mediated alternative activation of macrophage. Kim KH, Kim YH, Son JE, Lee JH, Kim S, Choe MS, Moon JH, Zhong J, Fu K, Lenglin F, Yoo JA, Bilan PJ, Klip A, Nagy A, Kim JR, Park JG, Hussein SM, Doh KO, Hui CC, Sung HK. Cell Research. 2017 Nov;27(11):1309-1326.
The two domain hypothesis of limb prepattern and its relevance to congenital limb anomalies. Tao H, Kawakami Y, Hui CC, Hopyan S. Wiley Interdisciplinary Review: Developmental Biology. 2017 Mar 20.
A genetic female mouse model with congenital genitourinary anomalies and adult stages of urinary incontinence. Akbari P, Fathollahi A, Mo R, Kavran M, Episalla N, Hui CC, Farhat WA, Hijaz AK. Neurourology & Urodynamics. 2017 Feb 28.
Suppressor of Fused Chaperones Gli Proteins To Generate Transcriptional Responses to Sonic Hedgehog Signaling. Zhang Z, Shen L, Law K, Zhang Z, Liu X, Hua H, Li S, Huang H, Yue S, Hui CC, Cheng SY. Molecular & Cellular Biology. 2017 Jan 19;37(3).

Adult Gli2+/-;Gli3Δ699/+ Male and Female Mice Display a Spectrum of Genital Malformation. He F, Akbari P, Mo R, Zhang JJ, Hui CC, Kim PC, Farhat WA. PLoS One. 2016 Nov 4;11(11):e0165958.

Tibial hemimelia associated with GLI3 truncation. Deimling S, Sotiropoulos C, Lau K, Chaudhry S, Sturgeon K, Kelley S, Narayanan U, Howard A, Hui CC, Hopyan S. Journal of Human Genetics. 2016 May;61(5):443-6.

Irx3 is required for postnatal maturation of the mouse ventricular conduction system. Kim KH, Rosen A, Hussein SM, Puviindran V, Korogyi AS, Chiarello C, Nagy A, Hui CC, Backx PH. Science Reports. 2016 Jan 20;6:19197.

FTO Obesity Variant and Adipocyte Browning in Humans. Claussnitzer M, Hui CC, Kellis M. New England Journal of Medicine. 2016 Jan 14;374(2):192-3.

FTO Obesity Variant Circuitry and Adipocyte Browning in Humans. Claussnitzer M, Dankel SN, Kim KH, Quon G, Meuleman W, Haugen C, Glunk V, Sousa IS, Beaudry JL, Puviindran V, Abdennur NA, Liu J, Svensson PA, Hsu YH, Drucker DJ, Mellgren G, Hui CC, Hauner H, Kellis M. New England Journal of Medicine. 2015 Sep 3;373(10):895-907.

Identification of GLI Mutations in Patients with Hirschsprung Disease That Disrupt Enteric Nervous System Development in Mice. Liu JA, Lai FP, Gui HS, Sham MH, Tam PK, Garcia-Barcelo MM, Hui CC, Ngan ES. Gastroenterology. 2015 Aug 7. pii: S0016-5085(15)01093-8.

Kv4.3-Encoded Fast Transient Outward Current Is Presented in Kv4.2 Knockout Mouse Cardiomyocytes. Liu J, Kim KH, Morales MJ, Heximer SP, Hui CC, Backx PH. PLoS One. 2015 Jul 21;10(7):e0133274.

BCC or not: Sufu keeps it in check. Yin WC, Li ZJ, Hui CC. Oncoscience. 2015 Feb 20;2(2):77-8. eCollection.

T396I Mutation of Mouse Sufu Reduces the Stability and Activity of Gli3 Repressor. Makino S, Zhulyn O, Mo R, Puviindran V, Zhang X, Murata T, Fukumura R, Ishitsuka Y, Kotaki H, Matsumaru D, Ishii S, Hui CC, Gondo Y. PLoS One. 2015 10(3):e0119455.

Sufu and Kif7 in limb patterning and development. Zhulyn O, Hui CC. Developmental Dynamics. 2015 244(3):468-78.

Ptch2 shares overlapping functions with Ptch1 in Smo regulation and limb development. Zhulyn O, Nieuwenhuis E, Liu YC, Angers S, Hui CC. Developmental Biology. 2015 Jan 15;397(2):191-202.

Differential regulation of Gli proteins by Sufu in the lung affects PDGF signaling and myofibroblast development. Lin C, Chen MH, Yao E, Song H, Gacayan R, Hui CC, Chuang PT. Developmental Biology. 2014 Aug 15;392(2):324-33.

The PPFIA1-PP2A protein complex promotes trafficking of Kif7 to the ciliary tip and Hedgehog signalling. Liu YC, Couzens AL, Deshwar AR, B McBroom-Cerajewski LD, Zhang X, Puviindran V, Scott IC, Gingras AC, Hui CC, Angers S. Science Signalling. 2014 Dec 9;7(355):ra117.

Obesity-associated variants within FTO form long-range functional connections with IRX3. Smemo S, Tena JJ, Kim KH, Gamazon ER, Sakabe NJ, Gómez-Marín C, Aneas I, Credidio FL, Sobreira DR, Wasserman NF, Lee JH, Puviindran V, Tam D, Shen M, Son JE, Vakili NA, Sung HK, Naranjo S, Acemel RD, Manzanares M, Nagy A, Cox NJ, Hui CC, Gomez-Skarmeta JL, Nóbrega MA. Nature. 2014 Mar 20;507(7492):371-5.

A switch from low to high Shh activity regulates establishment of limb progenitors and signaling centers. Zhulyn O, Li D, Deimling S, Vakili NA, Mo R, Puviindran V, Chen MH, Chuang PT, Hopyan S, Hui CC. Developmental Cell. 2014 Apr 28;29(2):241-9.

Formation of proximal and anterior limb skeleton requires early function of Irx3 and Irx5 and is negatively regulated by Shh signalling. Li D, Sakuma R, Vakili NA, Mo R, Puviindran V, Deimling S, Zhang X, Hopyan S, Hui CC. Developmental Cell. 2014 Apr 28;29(2):233-40.

Patched 1 and patched 2 redundancy has a key role in regulating epidermal differentiation. Adolphe C, Nieuwenhuis E, Villani R, Li ZJ, Kaur P, Hui CC, Wainwright BJ. Journal of Investigative Dermatology. 2014 Jul;134(7):1981-90.

Evasion of p53 and G2/M checkpoints are characteristic of Hh-driven basal cell carcinoma. Li ZJ, Mack SC, Mak TH, Angers S, Taylor MD, Hui CC. Oncogene. 2014 May 15;33(20):2674-80.

Silk gland factor-2, involved in fibroin gene transcription, consists of LIM homeodomain, LIM-interacting, and single-stranded DNA-binding proteins. Ohno K, Sawada J, Takiya S, Kimoto M, Matsumoto A, Tsubota T, Uchino K, Hui CC, Sezutsu H, Handa H, Suzuki Y. Journal of Biological Chemistry. 2013 Nov 1;288(44):31581-91.

Kif3a controls murine nephron number via GLI3 repressor, cell survival, and gene expression in a lineage-specific manner. Chi L, Galtseva A, Chen L, Mo R, Hui CC, Rosenblum ND. PLoS One. 2013 Jun 7;8(6):e65448.

Ter94 ATPase complex targets k11-linked ubiquitinated ci to proteasomes for partial degradation. Zhang Z, Lv X, Yin WC, Zhang X, Feng J, Wu W, Hui CC, Zhang L, Zhao Y. Developmental Cell. 2013 Jun 24;25(6):636-44.

Sonic Hedgehog Signaling and VACTERL Association. Ngan ES, Kim KH, Hui CC. Molecular Syndromology. 2013 Feb;4(1-2):32-45.

Antagonistic and cooperative actions of Kif7 and Sufu define graded intracellular Gli activities in Hedgehog signaling. Law KK, Makino S, Mo R, Zhang X, Puviindran V, Hui CC. PLoS One. 2012;7(11):e50193.

Kif7 regulates Gli2 through Sufu-dependent and -independent functions during skin development and tumorigenesis. Li ZJ, Nieuwenhuis E, Nien W, Zhang X, Zhang J, Puviindran V, Wainwright BJ, Kim PC, Hui CC. Development. 2012 Nov;139(22):4152-61.

Cooperative and antagonistic roles for Irx3 and Irx5 in cardiac morphogenesis and postnatal physiology. Gaborit N, Sakuma R, Wylie JN, Kim KH, Zhang SS, Hui CC, Bruneau BG. Development. 2012 Nov;139(21):4007-19.

Suppressor of fused (Sufu) mediates the effect of parathyroid hormone-like hormone (Pthlh) on chondrocyte differentiation in the growth plate. Hsu SH, Zhang X, Cheng S, Wunder JS, Hui CC, Alman BA. Journal of Biological Chemistry. 2012 Oct 19;287(43):36222-8.

A new Cre driver mouse line, Tcf21/Pod1-Cre, targets metanephric mesenchyme. Maezawa Y, Binnie M, Li C, Thorner P, Hui CC, Alman B, Taketo MM, Quaggin SE. PLoS One. 2012;7(7):e40547.

Iroquois homeodomain transcription factors in heart development and function. Kim KH, Rosen A, Bruneau BG, Hui CC, Backx PH. Circulation Research. 2012 May 25;110(11):1513-24.

Hedgehog/Notch-induced premature gliogenesis represents a new disease mechanism for Hirschsprung disease in mice and humans. Ngan ES, Garcia-Barceló MM, Yip BH, Poon HC, Lau ST, Kwok CK, Sat E, Sham MH, Wong KK, Wainwright BJ, Cherny SS, Hui CC, Sham PC, Lui VC, Tam PK. Journal of Clinical Investigation. 2011 Sep;121(9):3467-78.

Iroquois homeobox gene 3 establishes fast conduction in the cardiac His-Purkinje network. Zhang SS, Kim KH, Rosen A, Smyth JW, Sakuma R, Delgado-Olguín P, Davis M, Chi NC, Puviindran V, Gaborit N, Sukonnik T, Wylie JN, Brand-Arzamendi K, Farman GP, Kim J, Rose RA, Marsden PA, Zhu Y, Zhou YQ, Miquerol L, Henkelman RM, Stainier DY, Shaw RM, Hui CC, Bruneau BG, Backx PH. Proceedings of the National Academy of Sciences USA. 2011 Aug 16;108(33):13576-81.

Gli proteins in development and disease. Hui CC, Angers S. Annual Review of Cell & Developmental Biology. 2011;27:513-37.

Kif7 promotes hedgehog signaling in growth plate chondrocytes by restricting the inhibitory function of Sufu. Hsu SH, Zhang X, Yu C, Li ZJ, Wunder JS, Hui CC, Alman BA. Development. 2011 Sep;138(17):3791-801.

The role of sonic hedgehog-Gli2 pathway in the masculinization of external genitalia. Miyagawa S, Matsumaru D, Murashima A, Omori A, Satoh Y, Haraguchi R, Motoyama J, Iguchi T, Nakagata N, Hui CC, Yamada G. Endocrinology. 2011 Jul;152(7):2894-903.

Suppressor of fused is required to maintain the multipotency of neural progenitor cells in the retina. Cwinn MA, Mazerolle C, McNeill B, Ringuette R, Thurig S, Hui CC, Wallace VA. Journal of Neuroscience. 2011 Mar 30;31(13):5169-80.

Suppressor of fused controls mid-hindbrain patterning and cerebellar morphogenesis via GLI3 repressor. Kim JJ, Gill PS, Rotin L, van Eede M, Henkelman RM, Hui CC, Rosenblum ND. Journal of Neuroscience. 2011 Feb 2;31(5):1825-36.

Primordial germ cell proliferation is impaired in Fused Toes mutant embryos. Kim B, Kim Y, Sakuma R, Hui CC, Rüther U, Jorgensen JS. Developmental Biology. 2011 Jan 15;349(2):417-26.

A cascade of irx1a and irx2a controls shh expression during retinogenesis. Choy SW, Cheng CW, Lee ST, Li VW, Hui MN, Hui CC, Liu D, Cheng SH. Developmental Dynamics. 2010 Dec;239(12):3204-14.

Disruption at the PTCHD1 Locus on Xp22.11 in Autism spectrum disorder and intellectual disability. Noor A, Whibley A, Marshall CR, Gianakopoulos PJ, Piton A, Carson AR, Orlic-Milacic M, Lionel AC, Sato D, Pinto D, Drmic I, Noakes C, Senman L, Zhang X, Mo R, Gauthier J, Crosbie J, Pagnamenta AT, Munson J, Estes AM, Fiebig A, Franke A, Schreiber S, Stewart AF, Roberts R, McPherson R, Guter SJ, Cook EH Jr, Dawson G, Schellenberg GD, Battaglia A, Maestrini E; Autism Genome Project Consortium, Jeng L, Hutchison T, Rajcan-Separovic E, Chudley AE, Lewis SM, Liu X, Holden JJ, Fernandez B, Zwaigenbaum L, Bryson SE, Roberts W, Szatmari P, Gallagher L, Stratton MR, Gecz J, Brady AF, Schwartz CE, Schachar RJ, Monaco AP, Rouleau GA, Hui CC, Lucy Raymond F, Scherer SW, Vincent JB. Science Translational Medicine. 2010 Sep 15;2(49):49ra68.

Hedgehog regulates distinct vascular patterning events through VEGF-dependent and -independent mechanisms. Coultas L, Nieuwenhuis E, Anderson GA, Cabezas J, Nagy A, Henkelman RM, Hui CC, Rossant J. Blood. 2010 Jul 29;116(4):653-60.

Drosophila genome-wide obesity screen reveals hedgehog as a determinant of brown versus white adipose cell fate. Pospisilik JA, Schramek D, Schnidar H, Cronin SJ, Nehme NT, Zhang X, Knauf C, Cani PD, Aumayr K, Todoric J, Bayer M, Haschemi A, Puviindran V, Tar K, Orthofer M, Neely GG, Dietzl G, Manoukian A, Funovics M, Prager G, Wagner O, Ferrandon D, Aberger F, Hui CC, Esterbauer H, Penninger JM. Cell. 2010 Jan 8;140(1):148-60.

Lunatic Fringe-mediated Notch signaling is required for lung alveogenesis. Xu K, Nieuwenhuis E, Cohen BL, Wang W, Canty AJ, Danska JS, Coultas L, Rossant J, Wu MY, Piscione TD, Nagy A, Gossler A, Hicks GG, Hui CC, Henkelman RM, Yu LX, Sled JG, Gridley T, Egan SE. American Journal of Physiology – Lung Cellular and Molecular Physiology. 2010 Jan;298(1):L45-56.

GLI3 repressor controls nephron number via regulation of Wnt11 and Ret in ureteric tip cells. Cain JE, Islam E, Haxho F, Chen L, Bridgewater D, Nieuwenhuis E, Hui CC, Rosenblum ND. PLoS One. 2009 Oct 7;4(10):e7313.

Cilium-independent regulation of Gli protein function by Sufu in Hedgehog signaling is evolutionarily conserved. Chen MH, Wilson CW, Li YJ, Law KK, Lu CS, Gacayan R, Zhang X, Hui CC, Chuang PT. Genes & Development. 2009 Aug 15;23(16):1910-28.

The kinesin protein Kif7 is a critical regulator of Gli transcription factors in mammalian hedgehog signaling. Cheung HO, Zhang X, Ribeiro A, Mo R, Makino S, Puviindran V, Law KK, Briscoe J, Hui CC. Science Signalling. 2009 Jun 23;2(76):ra29.

Multipotent CD15+ cancer stem cells in patched-1-deficient mouse medulloblastoma. Ward RJ, Lee L, Graham K, Satkunendran T, Yoshikawa K, Ling E, Harper L, Austin R, Nieuwenhuis E, Clarke ID, Hui CC, Dirks PB. Cancer Research. 2009 Jun 1;69(11):4682-90.

Hedgehog signaling in development and cancer. Jiang J, Hui CC. Developmental Cell. 2008 Dec;15(6):801-12. Review article.

Sonic Hedgehog mediator Gli2 regulates bladder mesenchymal patterning. Cheng W, Yeung CK, Ng YK, Zhang JR, Hui CC, Kim PC. Journal of Urology. 2008 Oct;180(4):1543-50.

Genetic control of circuit function: Vsx1 and Irx5 transcription factors regulate contrast adaptation in the mouse retina. Kerschensteiner D, Liu H, Cheng CW, Demas J, Cheng SH, Hui CC, Chow RL, Wong RO. Journal of Neuroscience. 2008 Mar 5;28(10):2342-52.

Zebrafish homologue irx1a is required for the differentiation of serotonergic neurons. Cheng CW, Yan CH, Choy SW, Hui MN, Hui CC, Cheng SH. Developmental Dynamics. 2007 Sep;236(9):2661-7.

Epidermal hyperplasia and expansion of the interfollicular stem cell compartment in mutant mice with a C-terminal truncation of Patched1. Nieuwenhuis E, Barnfield PC, Makino S, Hui CC. Developmental Biology. 2007 Aug 15;308(2):547-60.

Gli2 and Gli3 play distinct roles in the dorsoventral patterning of the mouse hindbrain. Lebel M, Mo R, Shimamura K, Hui CC. Developmental Biology. 2007 Feb 1;302(1):345-55.

Dusty protein kinases: primary structure, gene evolution, tissue specific expression and unique features of the catalytic domain. Peng J, Dong W, Chen Y, Mo R, Cheng JF, Hui CC, Mohandas N, Huang CH. Biochimica et Biophysica Acta. 2006 Nov-Dec;1759(11-12):562-72.

Gli2 and Gli3 play distinct roles in the dorsoventral patterning of the mouse hindbrain. Lebel M, Mo R, Shimamura K, Hui CC. Developmental Biology. 2007 Feb 1;302(1):345-55.

Loss of the mouse ortholog of the shwachman-diamond syndrome gene (Sbds) results in early embryonic lethality. Zhang S, Shi M, Hui CC, Rommens JM. Molecular & Cellular Biology. 2006 Sep;26(17):6656-63.

Mice with a targeted mutation of patched2 are viable but develop alopecia and epidermal hyperplasia. Nieuwenhuis E, Motoyama J, Barnfield PC, Yoshikawa Y, Zhang X, Mo R, Crackower MA, Hui CC. Molecular & Cellular Biology. 2006 Sep;26(17):6609-22.

Fibroblast growth factor signals regulate a wave of Hedgehog activation that is essential for coronary vascular development. Lavine KJ, White AC, Park C, Smith CS, Choi K, Long F, Hui CC, Ornitz DM. Genes & Development. 2006 Jun 15;20(12):1651-66.

The homeobox gene irx1a is required for the propagation of the neurogenic waves in the zebrafish retina. Cheng CW, Yan CH, Hui CC, Strähle U, Cheng SH. Mechanisms of Development. 2006 Mar;123(3):252-63.

GLI3-dependent transcriptional repression of Gli1, Gli2 and kidney patterning genes disrupts renal morphogenesis. Hu MC, Mo R, Bhella S, Wilson CW, Chuang PT, Hui CC, Rosenblum ND. Development. 2006 Feb;133(3):569-78.

Cooperative and antagonistic interactions between Sall4 and Tbx5 pattern the mouse limb and heart. Koshiba-Takeuchi K, Takeuchi JK, Arruda EP, Kathiriya IS, Mo R, Hui CC, Srivastava D, Bruneau BG. Nature Genetics. 2006 Feb;38(2):175-83.

Negative regulation of Gli1 and Gli2 activator function by Suppressor of fused through multiple mechanisms. Barnfield PC, Zhang X, Thanabalasingham V, Yoshida M, Hui CC. Differentiation. 2005 Oct;73(8):397-405.

The homeodomain transcription factor Irx5 establishes the mouse cardiac ventricular repolarization gradient. Costantini DL, Arruda EP, Agarwal P, Kim KH, Zhu Y, Zhu W, Lebel M, Cheng CW, Park CY, Pierce SA, Guerchicoff A, Pollevick GD, Chan TY, Kabir MG, Cheng SH, Husain M, Antzelevitch C, Srivastava D, Gross GJ, Hui CC, Backx PH, Bruneau BG. Cell. 2005 Oct 21;123(2):347-58.

The Iroquois homeobox gene, Irx5, is required for retinal cone bipolar cell development. Cheng CW, Chow RL, Lebel M, Sakuma R, Cheung HO, Thanabalasingham V, Zhang X, Bruneau BG, Birch DG, Hui CC, McInnes RR, Cheng SH. Developmental Biology. 2005 Nov 1;287(1):48-60.

Pax9 and Jagged1 act downstream of Gli3 in vertebrate limb development. McGlinn E, van Bueren KL, Fiorenza S, Mo R, Poh AM, Forrest A, Soares MB, Bonaldo Mde F, Grimmond S, Hui CC, Wainwright B, Wicking C. Mechanisms of Development. 2005 Nov;122(11):1218-33.

Sox9 is essential for outer root sheath differentiation and the formation of the hair stem cell compartment. Vidal VP, Chaboissier MC, Lützkendorf S, Cotsarelis G, Mill P, Hui CC, Ortonne N, Ortonne JP, Schedl A. Current Biology. 2005 Aug 9;15(15):1340-51.

Shh controls epithelial proliferation via independent pathways that converge on N-Myc. Mill P, Mo R, Hu MC, Dagnino L, Rosenblum ND, Hui CC. Developmental Cell. 2005 Aug;9(2):293-303.

Angiotensin-converting enzyme 2 protects from severe acute lung failure. Imai Y, Kuba K, Rao S, Huan Y, Guo F, Guan B, Yang P, Sarao R, Wada T, Leong-Poi H, Crackower MA, Fukamizu A, Hui CC, Hein L, Uhlig S, Slutsky AS, Jiang C, Penninger JM. Nature. 2005 Jul 7;436(7047):112-6.

Tbx20 dose-dependently regulates transcription factor networks required for mouse heart and motoneuron development. Takeuchi JK, Mileikovskaia M, Koshiba-Takeuchi K, Heidt AB, Mori AD, Arruda EP, Gertsenstein M, Georges R, Davidson L, Mo R, Hui CC, Henkelman RM, Nemer M, Black BL, Nagy A, Bruneau BG. Development. 2005 May;132(10):2463-74.

Hedgehog signaling and congenital malformations. Nieuwenhuis E, Hui CC. Clinical Genetics. 2005 Mar;67(3):193-208. Review article.

Gli2 and Gli3 have redundant and context-dependent function in skeletal muscle formation. McDermott A, Gustafsson M, Elsam T, Hui CC, Emerson CP Jr, Borycki AG. Development. 2005 Jan;132(2):345-57.

A dermal niche for multipotent adult skin-derived precursor cells. Fernandes KJ, McKenzie IA, Mill P, Smith KM, Akhavan M, Barnabé-Heider F, Biernaskie J, Junek A, Kobayashi NR, Toma JG, Kaplan DR, Labosky PA, Rafuse V, Hui CC, Miller FD. Nature Cell Biology. 2004 Nov;6(11):1082-93.

Genomic imprinting of PPP1R9A encoding neurabin I in skeletal muscle and extra-embryonic tissues. Nakabayashi K, Makino S, Minagawa S, Smith AC, Bamforth JS, Stanier P, Preece M, Parker-Katiraee L, Paton T, Oshimura M, Mill P, Yoshikawa Y, Hui CC, Monk D, Moore GE, Scherer SW. Journal of Medical Genetics. 2004 Aug;41(8):601-8.

Failure of a medulloblastoma-derived mutant of SUFU to suppress WNT signaling. Taylor MD, Zhang X, Liu L, Hui CC, Mainprize TG, Scherer SW, Wainwright B, Hogg D, Rutka JT. Oncogene. 2004 Jun 3;23(26):4577-83.

Shh expression is required for embryonic hair follicle but not mammary gland development. Michno K, Boras-Granic K, Mill P, Hui CC, Hamel PA. Developmental Biology. 2003 Dec 1;264(1):153-65.

Interplays of Gli2 and Gli3 and their requirement in mediating Shh-dependent sclerotome induction. Buttitta L, Mo R, Hui CC, Fan CM. Development. 2003 Dec;130(25):6233-43.

The Iroquois homeobox gene Irx2 is not essential for normal development of the heart and midbrain-hindbrain boundary in mice. Lebel M, Agarwal P, Cheng CW, Kabir MG, Chan TY, Thanabalasingham V, Zhang X, Cohen DR, Husain M, Cheng SH, Bruneau BG, Hui CC. Molecular & Cellular Biology. 2003 Nov;23(22):8216-25.

Differential activities of Sonic hedgehog mediated by Gli transcription factors define distinct neuronal subtypes in the dorsal thalamus. Hashimoto-Torii K, Motoyama J, Hui CC, Kuroiwa A, Nakafuku M, Shimamura K. Mechanisms of Development. 2003 Oct;120(10):1097-111.

Cbl-3-deficient mice exhibit normal epithelial development. Griffiths EK, Sanchez O, Mill P, Krawczyk C, Hojilla CV, Rubin E, Nau MM, Khokha R, Lipkowitz S, Hui CC, Penninger JM. Molecular & Cellular Biology. 2003 Nov;23(21):7708-18.

Gli2 is required for normal Shh signaling and oligodendrocyte development in the spinal cord. Qi Y, Tan M, Hui CC, Qiu M. Molecular & Cellular Neuroscience. 2003 Jul;23(3):440-50.

Differential requirement for Gli2 and Gli3 in ventral neural cell fate specification. Motoyama J, Milenkovic L, Iwama M, Shikata Y, Scott MP, Hui CC. Developmental Biology. 2003 Jul 1;259(1):150-61.

Essential role of Fkbp6 in male fertility and homologous chromosome pairing in meiosis. Crackower MA, Kolas NK, Noguchi J, Sarao R, Kikuchi K, Kaneko H, Kobayashi E, Kawai Y, Kozieradzki I, Landers R, Mo R, Hui CC, Nieves E, Cohen PE, Osborne LR, Wada T, Kunieda T, Moens PB, Penninger JM. Science. 2003 May 23;300(5623):1291-5.

Notch1 functions as a tumor suppressor in mouse skin. Nicolas M, Wolfer A, Raj K, Kummer JA, Mill P, van Noort M, Hui CC, Clevers H, Dotto GP, Radtke F. Nature Genetics. 2003 Mar;33(3):416-21.

Sonic hedgehog-dependent activation of Gli2 is essential for embryonic hair follicle development. Mill P, Mo R, Fu H, Grachtchouk M, Kim PC, Dlugosz AA, Hui CC. Genes & Development. 2003 Jan 15;17(2):282-94.

Dissecting the oncogenic potential of Gli2: deletion of an NH(2)-terminal fragment alters skin tumor phenotype. Sheng H, Goich S, Wang A, Grachtchouk M, Lowe L, Mo R, Lin K, de Sauvage FJ, Sasaki H, Hui CC, Dlugosz AA. Cancer Research. 2002 Sep 15;62(18):5308-16.

Twist plays an essential role in FGF and SHH signal transduction during mouse limb development. O’Rourke MP, Soo K, Behringer RR, Hui CC, Tam PP. Developmental Biology. 2002 Aug 1;248(1):143-56.

Mutations in SUFU predispose to medulloblastoma. Taylor MD, Liu L, Raffel C, Hui CC, Mainprize TG, Zhang X, Agatep R, Chiappa S, Gao L, Lowrance A, Hao A, Goldstein AM, Stavrou T, Scherer SW, Dura WT, Wainwright B, Squire JA, Rutka JT, Hogg D. Nature Genetics. 2002 Jul;31(3):306-10.

Anorectal malformations caused by defects in sonic hedgehog signaling. Mo R, Kim JH, Zhang J, Chiang C, Hui CC, Kim PC. American Journal of Pathology. 2001 Aug;159(2):765-74.

Suppressor of fused negatively regulates beta-catenin signaling. Meng X, Poon R, Zhang X, Cheah A, Ding Q, Hui CC, Alman B. Journal of Biological Chemistry. 2001 Oct 26;276(43):40113-9.

The VACTERL association: lessons from the Sonic hedgehog pathway. Kim J, Kim P, Hui CC. Clinical Genetics. 2001 May;59(5):306-15. Review article.

Murine models of VACTERL syndrome: Role of sonic hedgehog signaling pathway. Kim PC, Mo R, Hui CC. Journal of Paediatric Surgery. 2001 Feb;36(2):381-4.

Evidence for the differential regulation of Nkx-6.1 expression in the ventral spinal cord and foregut by Shh-dependent and -independent mechanisms. Cai J, Xu X, Yin H, Wu R, Modderman G, Chen Y, Jensen J, Hui CC, Qiu M. Genesis. 2000 May;27(1):6-11.

Cloning and characterization of the murine glucosamine-6-phosphate acetyltransferase EMeg32. Differential expression and intracellular membrane association. Boehmelt G, Fialka I, Brothers G, McGinley MD, Patterson SD, Mo R, Hui CC, Chung S, Huber LA, Mak TW, Iscove NN. Journal of Biological Chemistry. 2000 Apr 28;275(17):12821-32.

Mouse Gli1 mutants are viable but have defects in SHH signaling in combination with a Gli2 mutation. Park HL, Bai C, Platt KA, Matise MP, Beeghly A, Hui CC, Nakashima M, Joyner AL. Development. 2000 Apr;127(8):1593-605.

Expression of two novel mouse Iroquois homeobox genes during neurogenesis. Cohen DR, Cheng CW, Cheng SH, Hui CC. Mechanisms of Development. 2000 Mar 1;91(1-2):317-21.

Basal cell carcinomas in mice overexpressing Gli2 in skin. Grachtchouk M, Mo R, Yu S, Zhang X, Sasaki H, Hui CC, Dlugosz AA. Nature Genetics. 2000 Mar;24(3):216-7.

New mouse models of congenital anorectal malformations. Kimmel SG, Mo R, Hui CC, Kim PC. Journal of Paediatric Surgery. 2000 Feb;35(2):227-30; discussion 230-1.

Developmentally regulated expression of the transcriptional cofactors/histone acetyltransferases CBP and p300 during mouse embryogenesis. Partanen A, Motoyama J, Hui CC. International Journal of Developmental Biology. 1999 Sep;43(6):487-94.

Mouse suppressor of fused is a negative regulator of sonic hedgehog signaling and alters the subcellular distribution of Gli1. Ding Q, Fukami Si, Meng X, Nishizaki Y, Zhang X, Sasaki H, Dlugosz A, Nakafuku M, Hui CC. Current Biology. 1999 Oct 7;9(19):1119-22.

The Shh signalling pathway in early tooth development. Hardcastle Z, Hui CC, Sharpe PT. Cellular & Molecular Biology. 1999 Jul;45(5):567-78.

Targeted overexpression of elafin protects mice against cardiac dysfunction and mortality following viral myocarditis. Zaidi SH, Hui CC, Cheah AY, You XM, Husain M, Rabinovitch M. Journal of Clinical Investigation. 1999 Apr;103(8):1211-9.

Essential function of Gli2 and Gli3 in the formation of lung, trachea and oesophagus. Motoyama J, Liu J, Mo R, Ding Q, Post M, Hui CC. Nature Genetics. 1998 Sep;20(1):54-7.

The Shh signalling pathway in tooth development: defects in Gli2 and Gli3 mutants. Hardcastle Z, Mo R, Hui CC, Sharpe PT. Development. 1998 Aug;125(15):2803-11.

Diminished Sonic hedgehog signaling and lack of floor plate differentiation in Gli2 mutant mice. Ding Q, Motoyama J, Gasca S, Mo R, Sasaki H, Rossant J, Hui CC. Development. 1998 Jul;125(14):2533-43.

Development of an epithelium-specific expression cassette with human DNA regulatory elements for transgene expression in lung airways. Chow YH, O’Brodovich H, Plumb J, Wen Y, Sohn KJ, Lu Z, Zhang F, Lukacs GL, Tanswell AK, Hui CC, Buchwald M, Hu J. Proceedings of the National Academy of Sciences USA. 1997 Dec 23;94(26):14695-700.

Activation of stress-activated protein kinases/c-Jun N-terminal protein kinases (SAPKs/JNKs) by a novel mitogen-activated protein kinase kinase. Yao Z, Diener K, Wang XS, Zukowski M, Matsumoto G, Zhou G, Mo R, Sasaki T, Nishina H, Hui CC, Tan TH, Woodgett JP, Penninger JM. Journal of Biological Chemistry. 1997 Dec 19;272(51):32378-83.

Fringe boundaries coincide with Notch-dependent patterning centres in mammals and alter Notch-dependent development in Drosophila. Cohen B, Bashirullah A, Dagnino L, Campbell C, Fisher WW, Leow CC, Whiting E, Ryan D, Zinyk D, Boulianne G, Hui CC, Gallie B, Phillips RA, Lipshitz HD, Egan SE. Nature Genetics. 1997 Jul;16(3):283-8.

Brca2 is required for embryonic cellular proliferation in the mouse. Suzuki A, de la Pompa JL, Hakem R, Elia A, Yoshida R, Mo R, Nishina H, Chuang T, Wakeham A, Itie A, Koo W, Billia P, Ho A, Fukumoto M, Hui CC, Mak TW. Genes & Development. 1997 May 15;11(10):1242-52.

The tumor suppressor gene Brca1 is required for embryonic cellular proliferation in the mouse. Hakem R, de la Pompa JL, Sirard C, Mo R, Woo M, Hakem A, Wakeham A, Potter J, Reitmair A, Billia F, Firpo E, Hui CC, Roberts J, Rossant J, Mak TW. Cell. 1996 Jun 28;85(7):1009-23.

Characterization of the split hand/split foot malformation locus SHFM1 at 7q21.3-q22.1 and analysis of a candidate gene for its expression during limb development. Crackower MA, Scherer SW, Rommens JM, Hui CC, Poorkaj P, Soder S, Cobben JM, Hudgins L, Evans JP, Tsui LC. Human Molecular Genetics. 1996 May;5(5):571-9.

A role for En-2 and other murine homologues of Drosophila segment polarity genes in regulating positional information in the developing cerebellum. Millen KJ, Hui CC, Joyner AL. Development. 1995 Dec;121(12):3935-45.

Presence of isl-1-related LIM domain homeobox genes in teleost and their similar patterns of expression in brain and spinal cord. Gong Z, Hui CC, Hew CL. Journal of Biological Chemistry. 1995 Feb 17;270(7):3335-45.

Expression of three mouse homologs of the Drosophila segment polarity gene cubitus interruptus, Gli, Gli-2, and Gli-3, in ectoderm- and mesoderm-derived tissues suggests multiple roles during postimplantation development. Hui CC, Slusarski D, Platt KA, Holmgren R, Joyner AL. Developmental Biology. 1994 Apr;162(2):402-13.

Molecular cloning of a POU domain-containing factor involved in the regulation of the Bombyx sericin-1 gene. Fukuta M, Matsuno K, Hui CC, Nagata T, Takiya S, Xu PX, Ueno K, Suzuki Y. Journal of Biological Chemistry. 1993 Sep 15;268(26):19471-5.

SH2-containing phosphotyrosine phosphatase as a target of protein-tyrosine kinases. Feng GS, Hui CC, Pawson T. Science. 1993 Mar 12;259(5101):1607-11.

A mouse model of greig cephalopolysyndactyly syndrome: the extra-toesJ mutation contains an intragenic deletion of the Gli3 gene. Hui CC, Joyner AL. Nature Genetics. 1993 Mar;3(3):241-6. Erratum in: Nat Genet 1998 Aug;19(4):404.

Molecular analysis of the deletion mutants in the E homeotic complex of the silkworm Bombyx mori. Ueno K, Hui CC, Fukuta M, Suzuki Y. Development. 1992 Mar;114(3):555-63.

Molecular characterization and silk gland expression of Bombyx engrailed and invected genes. Hui CC, Matsuno K, Ueno K, Suzuki Y. Proceedings of the National Academy of Sciences USA. 1992 Jan 1;89(1):167-71.

Fibroin gene promoter contains a cluster of homeodomain binding sites that interact with three silk gland factors. Hui CC, Matsuno K, Suzuki Y. Journal of Molecular Biology. 1990 Jun 20;213(4):651-70.

Homeodomain binding sites in the 5′ flanking region of the Bombyx mori silk fibroin light-chain gene. Hui CC, Suzuki Y, Kikuchi Y, Mizuno S. Journal of Molecular Biology. 1990 Jun 5;213(3):395-8.

Transcriptional stimulation via SC site of Bombyx sericin-1 gene through an interaction with a DNA binding protein SGF-3. Matsuno K, Takiya S, Hui CC, Suzuki T, Fukuta M, Ueno K, Suzuki Y. Nucleic Acids Research. 1990 Apr 11;18(7):1853-8.

Drosophila proliferating cell nuclear antigen (cyclin) gene: structure, expression during development, and specific binding of homeodomain proteins to its 5′-flanking region. Yamaguchi M, Nishida Y, Moriuchi T, Hirose F, Hui CC, Suzuki Y, Matsukage A. Molecular & Cellular Biology. 1990 Mar;10(3):872-9.

A contribution of the core-promoter and its surrounding regions to the preferential transcription of the fibroin gene in posterior silk gland extracts. Takiya S, Hui CC, Suzuki Y. EMBO Journal. 1990 Feb;9(2):489-96.

Enhancement of transcription from the Ad2 major late promoter by upstream elements of the fibroin- and sericin-1-encoding genes in silk gland extracts. Hui CC, Suzuki Y. Gene. 1989 Dec 28;85(2):403-11.

Transcription signals and protein binding sites for sericin gene transcription in vitro. Matsuno K, Hui CC, Takiya S, Suzuki T, Ueno K, Suzuki Y. Journal of Biological Chemistry. 1989 Nov 5;264(31):18707-13.

Molecular cloning of a gene coding for abundant transcript in red blood cells of the anemic rat. Hui CC, Cheng LY, Lam VM, Tam JW. Birth Defects Original Article Series. 1987;23(5A):139-45.

Hemoglobin switching: a new animal model for genetic and molecular studies. Tam JW, Hui CC, Woo C, Lam VM. Birth Defects Original Article Series. 1987;23(5A):133-7.