PubMed

Math1 is essential for the development of hindbrain neurons critical for perinatal breathing.

Rose MF, Ren J, Ahmad KA, Chao HT, Klisch TJ, Flora A, Greer JJ, Zoghbi HY.

Neuron. 2009 Nov 12;64(3):341-54.PMID: 19914183 [PubMed - in process]Related articles

Atoh1-lineal neurons are required for hearing and for the survival of neurons in the spiral ganglion and brainstem accessory auditory nuclei.

Maricich SM, Xia A, Mathes EL, Wang VY, Oghalai JS, Fritzsch B, Zoghbi HY.

J Neurosci. 2009 Sep 9;29(36):11123-33.PMID: 19741118 [PubMed - indexed for MEDLINE]Related articlesFree article

Merkel cells are essential for light-touch responses.

Maricich SM, Wellnitz SA, Nelson AM, Lesniak DR, Gerling GJ, Lumpkin EA, Zoghbi HY.

Science. 2009 Jun 19;324(5934):1580-2.PMID: 19541997 [PubMed - indexed for MEDLINE]Related articles

Phosphorylation of ATXN1 at Ser776 in the cerebellum.

Jorgensen ND, Andresen JM, Lagalwar S, Armstrong B, Stevens S, Byam CE, Duvick LA, Lai S, Jafar-Nejad P, Zoghbi HY, Clark HB, Orr HT.

J Neurochem. 2009 Jul;110(2):675-86. Epub 2009 May 15.PMID: 19500214 [PubMed - indexed for MEDLINE]Related articlesFree article

Mouse models of MeCP2 disorders share gene expression changes in the cerebellum and hypothalamus.

Ben-Shachar S, Chahrour M, Thaller C, Shaw CA, Zoghbi HY.

Hum Mol Genet. 2009 Jul 1;18(13):2431-42. Epub 2009 Apr 15.PMID: 19369296 [PubMed - indexed for MEDLINE]Related articlesFree article

Complex rearrangements in patients with duplications of MECP2 can occur by fork stalling and template switching.

Carvalho CM, Zhang F, Liu P, Patel A, Sahoo T, Bacino CA, Shaw C, Peacock S, Pursley A, Tavyev YJ, Ramocki MB, Nawara M, Obersztyn E, Vianna-Morgante AM, Stankiewicz P, Zoghbi HY, Cheung SW, Lupski JR.

Hum Mol Genet. 2009 Jun 15;18(12):2188-203. Epub 2009 Mar 26.PMID: 19324899 [PubMed - indexed for MEDLINE]Related articles

The yin and yang of MeCP2 phosphorylation.

Chao HT, Zoghbi HY.

Proc Natl Acad Sci U S A. 2009 Mar 24;106(12):4577-8. Epub 2009 Mar 17. No abstract available. PMID: 19293386 [PubMed - indexed for MEDLINE]Related articlesFree article

Rett syndrome: what do we know for sure?

Zoghbi HY.

Nat Neurosci. 2009 Mar;12(3):239-40. No abstract available. PMID: 19238181 [PubMed - indexed for MEDLINE]Related articles

Identification and subclassification of new Atoh1 derived cell populations during mouse spinal cord development.

Miesegaes GR, Klisch TJ, Thaller C, Ahmad KA, Atkinson RC, Zoghbi HY.

Dev Biol. 2009 Mar 15;327(2):339-51. Epub 2008 Dec 25.PMID: 19135992 [PubMed - indexed for MEDLINE]Related articlesFree article

Characterization of the zebrafish atxn1/axh gene family.

Carlson KM, Melcher L, Lai S, Zoghbi HY, Clark HB, Orr HT.

J Neurogenet. 2009;23(3):313-23. Epub 2008 Dec 10.PMID: 19085187 [PubMed - in process]Related articlesFree article

Pathogenic mechanisms of a polyglutamine-mediated neurodegenerative disease, spinocerebellar ataxia type 1.

Zoghbi HY, Orr HT.

J Biol Chem. 2009 Mar 20;284(12):7425-9. Epub 2008 Oct 28. Review.PMID: 18957430 [PubMed - indexed for MEDLINE]Related articles

Failure of neuronal homeostasis results in common neuropsychiatric phenotypes.

Ramocki MB, Zoghbi HY.

Nature. 2008 Oct 16;455(7215):912-8. Review.PMID: 18923513 [PubMed - indexed for MEDLINE]Related articlesFree article

Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress.

Fyffe SL, Neul JL, Samaco RC, Chao HT, Ben-Shachar S, Moretti P, McGill BE, Goulding EH, Sullivan E, Tecott LH, Zoghbi HY.

Neuron. 2008 Sep 25;59(6):947-58.PMID: 18817733 [PubMed - indexed for MEDLINE]Related articlesFree article

Genetic modifiers of MeCP2 function in Drosophila.

Cukier HN, Perez AM, Collins AL, Zhou Z, Zoghbi HY, Botas J.

PLoS Genet. 2008 Sep 5;4(9):e1000179.PMID: 18773074 [PubMed - indexed for MEDLINE]Related articlesFree article

miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.

Lee Y, Samaco RC, Gatchel JR, Thaller C, Orr HT, Zoghbi HY.

Nat Neurosci. 2008 Oct;11(10):1137-9. Epub 2008 Aug 31.PMID: 18758459 [PubMed - indexed for MEDLINE]Related articlesFree article

Spinocerebellar ataxia type 6 knockin mice develop a progressive neuronal dysfunction with age-dependent accumulation of mutant CaV2.1 channels.

Watase K, Barrett CF, Miyazaki T, Ishiguro T, Ishikawa K, Hu Y, Unno T, Sun Y, Kasai S, Watanabe M, Gomez CM, Mizusawa H, Tsien RW, Zoghbi HY.

Proc Natl Acad Sci U S A. 2008 Aug 19;105(33):11987-92. Epub 2008 Aug 7.PMID: 18687887 [PubMed - indexed for MEDLINE]Related articlesFree article

MeCP2, a key contributor to neurological disease, activates and represses transcription.

Chahrour M, Jung SY, Shaw C, Zhou X, Wong ST, Qin J, Zoghbi HY.

Science. 2008 May 30;320(5880):1224-9.PMID: 18511691 [PubMed - indexed for MEDLINE]Related articlesFree article

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.

Lim J, Crespo-Barreto J, Jafar-Nejad P, Bowman AB, Richman R, Hill DE, Orr HT, Zoghbi HY.

Nature. 2008 Apr 10;452(7188):713-8. Epub 2008 Mar 12.PMID: 18337722 [PubMed - indexed for MEDLINE]Related articlesFree article

A partial loss of function allele of methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome.

Samaco RC, Fryer JD, Ren J, Fyffe S, Chao HT, Sun Y, Greer JJ, Zoghbi HY, Neul JL.

Hum Mol Genet. 2008 Jun 15;17(12):1718-27. Epub 2008 Mar 4.PMID: 18321864 [PubMed - indexed for MEDLINE]Related articlesFree article

The insulin-like growth factor pathway is altered in spinocerebellar ataxia type 1 and type 7.

Gatchel JR, Watase K, Thaller C, Carson JP, Jafar-Nejad P, Shaw C, Zu T, Orr HT, Zoghbi HY.

Proc Natl Acad Sci U S A. 2008 Jan 29;105(4):1291-6. Epub 2008 Jan 23.PMID: 18216249 [PubMed - indexed for MEDLINE]Related articlesFree article

dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1.

Al-Ramahi I, Pérez AM, Lim J, Zhang M, Sorensen R, de Haro M, Branco J, Pulst SM, Zoghbi HY, Botas J.

PLoS Genet. 2007 Dec 28;3(12):e234. Epub 2007 Nov 16.PMID: 18166084 [PubMed - indexed for MEDLINE]Related articlesFree article

The story of Rett syndrome: from clinic to neurobiology.

Chahrour M, Zoghbi HY.

Neuron. 2007 Nov 8;56(3):422-37. Review.PMID: 17988628 [PubMed - indexed for MEDLINE]Related articles

MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number.

Chao HT, Zoghbi HY, Rosenmund C.

Neuron. 2007 Oct 4;56(1):58-65.PMID: 17920015 [PubMed - indexed for MEDLINE]Related articlesFree article

The E-protein Tcf4 interacts with Math1 to regulate differentiation of a specific subset of neuronal progenitors.

Flora A, Garcia JJ, Thaller C, Zoghbi HY.

Proc Natl Acad Sci U S A. 2007 Sep 25;104(39):15382-7. Epub 2007 Sep 18.PMID: 17878293 [PubMed - indexed for MEDLINE]Related articlesFree article

Cell-specific expression of wild-type MeCP2 in mouse models of Rett syndrome yields insight about pathogenesis.

Alvarez-Saavedra M, Sáez MA, Kang D, Zoghbi HY, Young JI.

Hum Mol Genet. 2007 Oct 1;16(19):2315-25. Epub 2007 Jul 17.PMID: 17635839 [PubMed - indexed for MEDLINE]Related articlesFree article

Intestine-specific ablation of mouse atonal homolog 1 (Math1) reveals a role in cellular homeostasis.

Shroyer NF, Helmrath MA, Wang VY, Antalffy B, Henning SJ, Zoghbi HY.

Gastroenterology. 2007 Jun;132(7):2478-88. Epub 2007 Mar 24.PMID: 17570220 [PubMed - indexed for MEDLINE]Related articles

The role of LANP and ataxin 1 in E4F-mediated transcriptional repression.

Cvetanovic M, Rooney RJ, Garcia JJ, Toporovskaya N, Zoghbi HY, Opal P.

EMBO Rep. 2007 Jul;8(7):671-7. Epub 2007 Jun 8.PMID: 17557114 [PubMed - indexed for MEDLINE]Related articlesFree article

Hsp70/Hsc70 regulates the effect phosphorylation has on stabilizing ataxin-1.

Jorgensen ND, Andresen JM, Pitt JE, Swenson MA, Zoghbi HY, Orr HT.

J Neurochem. 2007 Sep;102(6):2040-8. Epub 2007 Jun 1.PMID: 17540008 [PubMed - indexed for MEDLINE]Related articles

Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model.

Watase K, Gatchel JR, Sun Y, Emamian E, Atkinson R, Richman R, Mizusawa H, Orr HT, Shaw C, Zoghbi HY.

PLoS Med. 2007 May;4(5):e182.PMID: 17535104 [PubMed - indexed for MEDLINE]Related articlesFree article

Trinucleotide repeat disorders.

Orr HT, Zoghbi HY.

Annu Rev Neurosci. 2007;30:575-621. Review.PMID: 17417937 [PubMed - indexed for MEDLINE]Related articles

Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.

Bowman AB, Lam YC, Jafar-Nejad P, Chen HK, Richman R, Samaco RC, Fryer JD, Kahle JJ, Orr HT, Zoghbi HY.

Nat Genet. 2007 Mar;39(3):373-9. Epub 2007 Feb 18.PMID: 17322884 [PubMed - indexed for MEDLINE]Related articles

ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology.

Lam YC, Bowman AB, Jafar-Nejad P, Lim J, Richman R, Fryer JD, Hyun ED, Duvick LA, Orr HT, Botas J, Zoghbi HY.

Cell. 2006 Dec 29;127(7):1335-47.PMID: 17190598 [PubMed - indexed for MEDLINE]Related articles

Increased MECP2 gene copy number as the result of genomic duplication in neurodevelopmentally delayed males.

del Gaudio D, Fang P, Scaglia F, Ward PA, Craigen WJ, Glaze DG, Neul JL, Patel A, Lee JA, Irons M, Berry SA, Pursley AA, Grebe TA, Freedenberg D, Martin RA, Hsich GE, Khera JR, Friedman NR, Zoghbi HY, Eng CM, Lupski JR, Beaudet AL, Cheung SW, Roa BB.

Genet Med. 2006 Dec;8(12):784-92.PMID: 17172942 [PubMed - indexed for MEDLINE]Related articles

RORalpha-mediated Purkinje cell development determines disease severity in adult SCA1 mice.

Serra HG, Duvick L, Zu T, Carlson K, Stevens S, Jorgensen N, Lysholm A, Burright E, Zoghbi HY, Clark HB, Andresen JM, Orr HT.

Cell. 2006 Nov 17;127(4):697-708.PMID: 17110330 [PubMed - indexed for MEDLINE]Related articles

Enhanced anxiety and stress-induced corticosterone release are associated with increased Crh expression in a mouse model of Rett syndrome.

McGill BE, Bundle SF, Yaylaoglu MB, Carson JP, Thaller C, Zoghbi HY.

Proc Natl Acad Sci U S A. 2006 Nov 28;103(48):18267-72. Epub 2006 Nov 15.PMID: 17108082 [PubMed - indexed for MEDLINE]Related articlesFree article

Huntingtin's critical cleavage.

Fryer JD, Zoghbi HY.

Nat Neurosci. 2006 Sep;9(9):1088-9. No abstract available. PMID: 16936769 [PubMed - indexed for MEDLINE]Related articles

Getting back to basics.

Maricich SM, Zoghbi HY.

Cell. 2006 Jul 14;126(1):11-5.PMID: 16839867 [PubMed - indexed for MEDLINE]Related articles

CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation.

Al-Ramahi I, Lam YC, Chen HK, de Gouyon B, Zhang M, Pérez AM, Branco J, de Haro M, Patterson C, Zoghbi HY, Botas J.

J Biol Chem. 2006 Sep 8;281(36):26714-24. Epub 2006 Jul 10.PMID: 16831871 [PubMed - indexed for MEDLINE]Related articlesFree article

A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration.

Lim J, Hao T, Shaw C, Patel AJ, Szabó G, Rual JF, Fisk CJ, Li N, Smolyar A, Hill DE, Barabási AL, Vidal M, Zoghbi HY.

Cell. 2006 May 19;125(4):801-14.PMID: 16713569 [PubMed - indexed for MEDLINE]Related articles

MeCP2 dysfunction in Rett syndrome and related disorders.

Moretti P, Zoghbi HY.

Curr Opin Genet Dev. 2006 Jun;16(3):276-81. Epub 2006 May 2. Review.PMID: 16647848 [PubMed - indexed for MEDLINE]Related articles

Glutamine-expanded ataxin-7 alters TFTC/STAGA recruitment and chromatin structure leading to photoreceptor dysfunction.

Helmlinger D, Hardy S, Abou-Sleymane G, Eberlin A, Bowman AB, Gansmüller A, Picaud S, Zoghbi HY, Trottier Y, Tora L, Devys D.

PLoS Biol. 2006 Mar;4(3):e67. Epub 2006 Feb 28.PMID: 16494529 [PubMed - indexed for MEDLINE]Related articlesFree article

Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome.

Moretti P, Levenson JM, Battaglia F, Atkinson R, Teague R, Antalffy B, Armstrong D, Arancio O, Sweatt JD, Zoghbi HY.

J Neurosci. 2006 Jan 4;26(1):319-27.PMID: 16399702 [PubMed - indexed for MEDLINE]Related articlesFree article

SILencing misbehaving proteins.

Zoghbi HY.

Nat Genet. 2005 Dec;37(12):1302-3. No abstract available. PMID: 16314860 [PubMed - indexed for MEDLINE]Related articles

Regulation of RNA splicing by the methylation-dependent transcriptional repressor methyl-CpG binding protein 2.

Young JI, Hong EP, Castle JC, Crespo-Barreto J, Bowman AB, Rose MF, Kang D, Richman R, Johnson JM, Berget S, Zoghbi HY.

Proc Natl Acad Sci U S A. 2005 Dec 6;102(49):17551-8. Epub 2005 Oct 26. Erratum in: Proc Natl Acad Sci U S A. 2006 Jan 31;103(5):1656. PMID: 16251272 [PubMed - indexed for MEDLINE]Related articlesFree article

Gfi1 functions downstream of Math1 to control intestinal secretory cell subtype allocation and differentiation.

Shroyer NF, Wallis D, Venken KJ, Bellen HJ, Zoghbi HY.

Genes Dev. 2005 Oct 15;19(20):2412-7.PMID: 16230531 [PubMed - indexed for MEDLINE]Related articlesFree article

MeCP2 dysfunction in humans and mice.

Zoghbi HY.

J Child Neurol. 2005 Sep;20(9):736-40. Review.PMID: 16225828 [PubMed - indexed for MEDLINE]Related articles

Diseases of unstable repeat expansion: mechanisms and common principles.

Gatchel JR, Zoghbi HY.

Nat Rev Genet. 2005 Oct;6(10):743-55. Review.PMID: 16205714 [PubMed - indexed for MEDLINE]Related articles

Math1 expression redefines the rhombic lip derivatives and reveals novel lineages within the brainstem and cerebellum.

Wang VY, Rose MF, Zoghbi HY.

Neuron. 2005 Oct 6;48(1):31-43.PMID: 16202707 [PubMed - indexed for MEDLINE]Related articles

Towards a proteome-scale map of the human protein-protein interaction network.

Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M.

Nature. 2005 Oct 20;437(7062):1173-8. Epub 2005 Sep 28.PMID: 16189514 [PubMed - indexed for MEDLINE]Related articles

The AXH domain of Ataxin-1 mediates neurodegeneration through its interaction with Gfi-1/Senseless proteins.

Tsuda H, Jafar-Nejad H, Patel AJ, Sun Y, Chen HK, Rose MF, Venken KJ, Botas J, Orr HT, Bellen HJ, Zoghbi HY.

Cell. 2005 Aug 26;122(4):633-44.PMID: 16122429 [PubMed - indexed for MEDLINE]Related articles