Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice

Nature Genetics

Volumn 27, Issue 3, 2001, Pages 327-331

  Chen, Richard Z ^a   Akbarian, Schahram ^a,b   Tudor, Matthew ^a,c   Jaenisch, Rudolf ^a,c  

^a WHITEHEAD INSTITUTE FOR BIOMEDICAL RESEARCH   (United States)

^b MASSACHUSETTS GENERAL HOSPITAL   (United States)

^c MASSACHUSETTS INSTITUTE OF TECHNOLOGY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BINDING PROTEIN; NUCLEAR PROTEIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN WEIGHT; CONTROLLED STUDY; CPG ISLAND; DNA METHYLATION; FEMALE; GENE EXPRESSION REGULATION; MALE; MENTAL DEFICIENCY; MOUSE; NERVE CELL; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN DEFICIENCY; RETT SYNDROME; X CHROMOSOME LINKAGE;

ANIMALS; CELL DIFFERENTIATION; CELL SIZE; CENTRAL NERVOUS SYSTEM; CHROMOSOMAL PROTEINS, NON-HISTONE; CPG ISLANDS; DISEASE MODELS, ANIMAL; DNA-BINDING PROTEINS; FEMALE; HUMANS; MALE; METHYL-CPG-BINDING PROTEIN 2; MICE; MICE, INBRED BALB C; MICE, INBRED C57BL; NEURONS; PHENOTYPE; REPRESSOR PROTEINS; RETT SYNDROME;

ANIMALIA; MAMMALIA;

EID: 0035093830     PISSN: 10614036     EISSN: None     Source Type: Journal    
DOI: 10.1038/85906     Document Type: Article

References (29)

1. Purification, sequence, and cellular localization of a novel chromosomal protein that binds to methylated DNA
2. Transcriptional repression by the methyl-CpG-binding protein MeCP2 involves a histone deacetylase complex
3. Methylated DNA and MeCP2 recruit histone deacetylase to repress transcription
4. A complex pattern of evolutionary conservation and alternative polyadenylation within the long 3- untranslated region of the methyl-CpG-binding protein 2 gene (MeCP2) suggests a regulatory role in gene expression
5. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2
6. Rett syndrome and beyond: Recurrent spontaneous and familial MECP2 mutations at CpG hotspots
7. Methyl-CpG-binding protein 2 mutations in Rett syndrome
8. Rett syndrome: A surprising result of mutation in MECP2
9. Uber ein eigenartiges hirnatrophisches Syndrom bei Hyperammonamie im Kindersalter
10. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: Report of 35 cases
11. Review of Rett syndrome
12. The methyl-CpG binding protein MeCP2 is essential for embryonic development in the mouse
13. Rett syndrome: Clinical update and review of recent genetic advances
14. Neonatal encephalopathy in two boys in families with recurrent Rett syndrome
15. A severely affected male born into a Rett syndrome kindred supports X-linked inheritance and allows extension of the exclusion map
16. Two affected boys in a Rett syndrome family: Clinical and molecular findings
17. A mutation in the rett syndrome gene, MECP2, causes X-linked mental retardation and progressive spasticity in males
18. Somatic mutation in MECP2 as a non-fatal neurodevelopmental disorder in males
19. MECP2 mutation in male patients with non-specific X-linked mental retardation
20. Cremediated gene inactivation demonstrates that FGF8 is required for cell survival and patterning of the first branchial arch
21. DNA hypomethylation perturbs the function and survival of CNS neurons in postnatal animals
22. A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome
23. Neuropathology of Rett syndrome
24. Head growth in Rett syndrome
25. Microscopic observations of the brain in Rett syndrome
26. Pervasive neuroanatomic abnormalities of the brain in three cases of Rett's syndrome
27. Essential role for TrkB receptors in hippocampus-mediated learning
28. Generalized lacz expression with the ROSA26 Cre reporter strain
29. CaMKllα·Cre transgene expression and recombination patterns in the mouse brain