Connection

HUDA ZOGHBI to Rett Syndrome

This is a "connection" page, showing publications HUDA ZOGHBI has written about Rett Syndrome.
Connection Strength

17.439
  1. A novel pathogenic mutation of MeCP2 impairs chromatin association independent of protein levels. Genes Dev. 2023 10 01; 37(19-20):883-900.
    View in: PubMed
    Score: 0.700
  2. MeCP2 regulates Gdf11, a dosage-sensitive gene critical for neurological function. Elife. 2023 02 27; 12.
    View in: PubMed
    Score: 0.672
  3. A weakened recurrent circuit in the hippocampus of Rett syndrome mice disrupts long-term memory representations. Neuron. 2022 05 18; 110(10):1689-1699.e6.
    View in: PubMed
    Score: 0.629
  4. Presymptomatic training mitigates functional deficits in a mouse model of Rett syndrome. Nature. 2021 04; 592(7855):596-600.
    View in: PubMed
    Score: 0.588
  5. Identification and characterization of conserved noncoding cis-regulatory elements that impact Mecp2 expression and neurological functions. Genes Dev. 2021 04 01; 35(7-8):489-494.
    View in: PubMed
    Score: 0.587
  6. Deleting Mecp2 from the cerebellum rather than its neuronal subtypes causes a delay in motor learning in mice. Elife. 2021 01 26; 10.
    View in: PubMed
    Score: 0.581
  7. Advances in understanding of Rett syndrome and MECP2 duplication syndrome: prospects for future therapies. Lancet Neurol. 2020 08; 19(8):689-698.
    View in: PubMed
    Score: 0.562
  8. Intellectual and Developmental Disabilities Research Centers: A Multidisciplinary Approach to Understand the Pathogenesis of Methyl-CpG Binding Protein 2-related Disorders. Neuroscience. 2020 10 01; 445:190-206.
    View in: PubMed
    Score: 0.552
  9. Losing Dnmt3a dependent methylation in inhibitory neurons impairs neural function by a mechanism impacting Rett syndrome. Elife. 2020 03 11; 9.
    View in: PubMed
    Score: 0.547
  10. The distinct methylation landscape of maturing neurons and its role in Rett syndrome pathogenesis. Curr Opin Neurobiol. 2019 12; 59:180-188.
    View in: PubMed
    Score: 0.529
  11. Rett Syndrome and the Ongoing Legacy of Close Clinical Observation. Cell. 2016 Oct 06; 167(2):293-297.
    View in: PubMed
    Score: 0.431
  12. Loss and Gain of MeCP2 Cause Similar Hippocampal Circuit Dysfunction that Is Rescued by Deep Brain Stimulation in a Rett Syndrome Mouse Model. Neuron. 2016 Aug 17; 91(4):739-747.
    View in: PubMed
    Score: 0.426
  13. Restoration of Mecp2 expression in GABAergic neurons is sufficient to rescue multiple disease features in a mouse model of Rett syndrome. Elife. 2016 06 21; 5.
    View in: PubMed
    Score: 0.423
  14. Loss of MeCP2 in Parvalbumin-and Somatostatin-Expressing Neurons in Mice Leads to Distinct Rett Syndrome-like Phenotypes. Neuron. 2015 Nov 18; 88(4):651-8.
    View in: PubMed
    Score: 0.406
  15. Karyopherin a 3 and karyopherin a 4 proteins mediate the nuclear import of methyl-CpG binding protein 2. J Biol Chem. 2015 Sep 11; 290(37):22485-93.
    View in: PubMed
    Score: 0.398
  16. MECP2 disorders: from the clinic to mice and back. J Clin Invest. 2015 Aug 03; 125(8):2914-23.
    View in: PubMed
    Score: 0.398
  17. Rett syndrome: disruption of epigenetic control of postnatal neurological functions. Hum Mol Genet. 2015 Oct 15; 24(R1):R10-6.
    View in: PubMed
    Score: 0.393
  18. MeCP2 binds to non-CG methylated DNA as neurons mature, influencing transcription and the timing of onset for Rett syndrome. Proc Natl Acad Sci U S A. 2015 Apr 28; 112(17):5509-14.
    View in: PubMed
    Score: 0.389
  19. Rett-causing mutations reveal two domains critical for MeCP2 function and for toxicity in MECP2 duplication syndrome mice. Elife. 2014 Jun 26; 3.
    View in: PubMed
    Score: 0.368
  20. An AT-hook domain in MeCP2 determines the clinical course of Rett syndrome and related disorders. Cell. 2013 Feb 28; 152(5):984-96.
    View in: PubMed
    Score: 0.336
  21. Adult neural function requires MeCP2. Science. 2011 Jul 08; 333(6039):186.
    View in: PubMed
    Score: 0.298
  22. Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes. Nature. 2010 Nov 11; 468(7321):263-9.
    View in: PubMed
    Score: 0.286
  23. Mouse models of MeCP2 disorders share gene expression changes in the cerebellum and hypothalamus. Hum Mol Genet. 2009 Jul 01; 18(13):2431-42.
    View in: PubMed
    Score: 0.257
  24. Rett syndrome: what do we know for sure? Nat Neurosci. 2009 Mar; 12(3):239-40.
    View in: PubMed
    Score: 0.255
  25. The story of Rett syndrome: from clinic to neurobiology. Neuron. 2007 Nov 08; 56(3):422-37.
    View in: PubMed
    Score: 0.233
  26. Enhanced anxiety and stress-induced corticosterone release are associated with increased Crh expression in a mouse model of Rett syndrome. Proc Natl Acad Sci U S A. 2006 Nov 28; 103(48):18267-72.
    View in: PubMed
    Score: 0.217
  27. MeCP2 dysfunction in Rett syndrome and related disorders. Curr Opin Genet Dev. 2006 Jun; 16(3):276-81.
    View in: PubMed
    Score: 0.209
  28. Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome. J Neurosci. 2006 Jan 04; 26(1):319-27.
    View in: PubMed
    Score: 0.205
  29. MeCP2 dysfunction in humans and mice. J Child Neurol. 2005 Sep; 20(9):736-40.
    View in: PubMed
    Score: 0.200
  30. Comprehensive assessment reveals numerous clinical and neurophysiological differences between MECP2-allelic disorders. Ann Clin Transl Neurol. 2025 Feb; 12(2):433-447.
    View in: PubMed
    Score: 0.192
  31. Acute MeCP2 loss in adult mice reveals transcriptional and chromatin changes that precede neurological dysfunction and inform pathogenesis. Neuron. 2025 Feb 05; 113(3):380-395.e8.
    View in: PubMed
    Score: 0.190
  32. Abnormalities of social interactions and home-cage behavior in a mouse model of Rett syndrome. Hum Mol Genet. 2005 Jan 15; 14(2):205-20.
    View in: PubMed
    Score: 0.189
  33. Modeling antisense oligonucleotide therapy in MECP2 duplication syndrome human iPSC-derived neurons reveals gene expression programs responsive to MeCP2 levels. Hum Mol Genet. 2024 11 08; 33(22):1986-2001.
    View in: PubMed
    Score: 0.189
  34. Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. Hum Mol Genet. 2004 Nov 01; 13(21):2679-89.
    View in: PubMed
    Score: 0.187
  35. Rett syndrome: a prototypical neurodevelopmental disorder. Neuroscientist. 2004 Apr; 10(2):118-28.
    View in: PubMed
    Score: 0.181
  36. X-chromosome inactivation patterns are unbalanced and affect the phenotypic outcome in a mouse model of rett syndrome. Am J Hum Genet. 2004 Mar; 74(3):511-20.
    View in: PubMed
    Score: 0.180
  37. Postnatal neurodevelopmental disorders: meeting at the synapse? Science. 2003 Oct 31; 302(5646):826-30.
    View in: PubMed
    Score: 0.176
  38. Rett syndrome and MeCP2: linking epigenetics and neuronal function. Am J Hum Genet. 2002 Dec; 71(6):1259-72.
    View in: PubMed
    Score: 0.165
  39. Balanced X chromosome inactivation patterns in the Rett syndrome brain. Am J Med Genet. 2002 Aug 01; 111(2):164-8.
    View in: PubMed
    Score: 0.161
  40. Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3. Neuron. 2002 Jul 18; 35(2):243-54.
    View in: PubMed
    Score: 0.161
  41. Insight into Rett syndrome: MeCP2 levels display tissue- and cell-specific differences and correlate with neuronal maturation. Hum Mol Genet. 2002 Jan 15; 11(2):115-24.
    View in: PubMed
    Score: 0.155
  42. Introduction: Rett syndrome. Ment Retard Dev Disabil Res Rev. 2002; 8(2):59-60.
    View in: PubMed
    Score: 0.155
  43. Genetic basis of Rett syndrome. Ment Retard Dev Disabil Res Rev. 2002; 8(2):82-6.
    View in: PubMed
    Score: 0.155
  44. Mutations in the gene encoding methyl-CpG-binding protein 2 cause Rett syndrome. Brain Dev. 2001 Dec; 23 Suppl 1:S147-51.
    View in: PubMed
    Score: 0.154
  45. Molecular genetics of Rett syndrome and clinical spectrum of MECP2 mutations. Curr Opin Neurol. 2001 Apr; 14(2):171-6.
    View in: PubMed
    Score: 0.147
  46. Methyl-CpG-binding protein 2 mutations in Rett syndrome. Curr Opin Genet Dev. 2000 Jun; 10(3):275-9.
    View in: PubMed
    Score: 0.139
  47. Influence of mutation type and X chromosome inactivation on Rett syndrome phenotypes. Ann Neurol. 2000 May; 47(5):670-9.
    View in: PubMed
    Score: 0.138
  48. Candidate gene analysis in Rett syndrome and the identification of 21 SNPs in Xq. Am J Med Genet. 2000 Jan 03; 90(1):69-71.
    View in: PubMed
    Score: 0.135
  49. Rett syndrome: methyl-CpG-binding protein 2 mutations and phenotype-genotype correlations. Am J Med Genet. 2000; 97(2):147-52.
    View in: PubMed
    Score: 0.135
  50. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet. 1999 Oct; 23(2):185-8.
    View in: PubMed
    Score: 0.133
  51. Impaired spatial memory codes in a mouse model of Rett syndrome. Elife. 2018 07 20; 7.
    View in: PubMed
    Score: 0.122
  52. Mutation analysis of the M6b gene in patients with Rett syndrome. Am J Med Genet. 1998 Jun 30; 78(2):165-8.
    View in: PubMed
    Score: 0.122
  53. Analysis of the genomic structure of the human glycine receptor alpha2 subunit gene and exclusion of this gene as a candidate for Rett syndrome. Am J Med Genet. 1998 Jun 30; 78(2):176-8.
    View in: PubMed
    Score: 0.122
  54. Genomic structure of a human holocytochrome c-type synthetase gene in Xp22.3 and mutation analysis in patients with Rett syndrome. Am J Med Genet. 1998 Jun 30; 78(2):179-81.
    View in: PubMed
    Score: 0.122
  55. Forniceal deep brain stimulation induces gene expression and splicing changes that promote neurogenesis and plasticity. Elife. 2018 03 23; 7.
    View in: PubMed
    Score: 0.119
  56. Forniceal deep brain stimulation rescues hippocampal memory in Rett syndrome mice. Nature. 2015 Oct 15; 526(7573):430-4.
    View in: PubMed
    Score: 0.101
  57. Brief report: MECP2 mutations in people without Rett syndrome. J Autism Dev Disord. 2014 Mar; 44(3):703-11.
    View in: PubMed
    Score: 0.090
  58. Isolation of a yeast artificial chromosome contig spanning the X chromosomal translocation breakpoint in a patient with Rett syndrome. Am J Med Genet. 1993 Nov 15; 47(7):1124-34.
    View in: PubMed
    Score: 0.088
  59. Scientists. Curiosity and observation. Biotechniques. 2013 Aug; 55(2):53.
    View in: PubMed
    Score: 0.086
  60. Preclinical research in Rett syndrome: setting the foundation for translational success. Dis Model Mech. 2012 Nov; 5(6):733-45.
    View in: PubMed
    Score: 0.082
  61. Examination of X chromosome markers in Rett syndrome: exclusion mapping with a novel variation on multilocus linkage analysis. Am J Hum Genet. 1992 Feb; 50(2):278-87.
    View in: PubMed
    Score: 0.078
  62. For Huda Zoghbi, collaboration is the key to unlocking the secrets of neurobiology. Interview by Kathryn Claiborn. J Clin Invest. 2011 Sep; 121(9):3364.
    View in: PubMed
    Score: 0.076
  63. A de novo X;3 translocation in Rett syndrome. Am J Med Genet. 1990 Jan; 35(1):148-51.
    View in: PubMed
    Score: 0.067
  64. Patterns of X chromosome inactivation in the Rett syndrome. Brain Dev. 1990; 12(1):131-5.
    View in: PubMed
    Score: 0.067
  65. MeCP2, a key contributor to neurological disease, activates and represses transcription. Science. 2008 May 30; 320(5880):1224-9.
    View in: PubMed
    Score: 0.060
  66. Specific mutations in methyl-CpG-binding protein 2 confer different severity in Rett syndrome. Neurology. 2008 Apr 15; 70(16):1313-21.
    View in: PubMed
    Score: 0.060
  67. A partial loss of function allele of methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome. Hum Mol Genet. 2008 Jun 15; 17(12):1718-27.
    View in: PubMed
    Score: 0.059
  68. Cell-specific expression of wild-type MeCP2 in mouse models of Rett syndrome yields insight about pathogenesis. Hum Mol Genet. 2007 Oct 01; 16(19):2315-25.
    View in: PubMed
    Score: 0.057
  69. Regulation of RNA splicing by the methylation-dependent transcriptional repressor methyl-CpG binding protein 2. Proc Natl Acad Sci U S A. 2005 Dec 06; 102(49):17551-8.
    View in: PubMed
    Score: 0.050
  70. Mutations in exon 1 of MECP2 are a rare cause of Rett syndrome. J Med Genet. 2005 Feb; 42(2):e15.
    View in: PubMed
    Score: 0.048
  71. A mutant form of MeCP2 protein associated with human Rett syndrome cannot be displaced from methylated DNA by notch in Xenopus embryos. Mol Cell. 2003 Aug; 12(2):425-35.
    View in: PubMed
    Score: 0.043
  72. Modelling brain diseases in mice: the challenges of design and analysis. Nat Rev Genet. 2003 Apr; 4(4):296-307.
    View in: PubMed
    Score: 0.042
  73. Diagnostic testing for Rett syndrome by DHPLC and direct sequencing analysis of the MECP2 gene: identification of several novel mutations and polymorphisms. Am J Hum Genet. 2000 Dec; 67(6):1428-36.
    View in: PubMed
    Score: 0.036
  74. Rett syndrome and beyond: recurrent spontaneous and familial MECP2 mutations at CpG hotspots. Am J Hum Genet. 1999 Dec; 65(6):1520-9.
    View in: PubMed
    Score: 0.034
  75. Mecp2 Deletion from Cholinergic Neurons Selectively Impairs Recognition Memory and Disrupts Cholinergic Modulation of the Perirhinal Cortex. eNeuro. 2019 Nov/Dec; 6(6).
    View in: PubMed
    Score: 0.033
  76. Apparent bias toward long gene misregulation in MeCP2 syndromes disappears after controlling for baseline variations. Nat Commun. 2018 08 13; 9(1):3225.
    View in: PubMed
    Score: 0.031
  77. A new Rett syndrome family consistent with X-linked inheritance expands the X chromosome exclusion map. Am J Hum Genet. 1997 Sep; 61(3):634-41.
    View in: PubMed
    Score: 0.029
  78. Rett syndrome: controlled study of an oral opiate antagonist, naltrexone. Ann Neurol. 1994 Apr; 35(4):464-70.
    View in: PubMed
    Score: 0.023
Connection Strength

The connection strength for concepts is the sum of the scores for each matching publication.

Publication scores are based on many factors, including how long ago they were written and whether the person is a first or senior author.