By Hook or by Crook: Multifaceted DNA-Binding Properties of MeCP2
نویسندگان
چکیده
Two new studies reveal novel DNA-binding properties of MeCP2, mutations of which cause Rett syndrome. Baker et al. report critical roles for the AT-hook domain of MeCP2 in chromatin organization and clinical features of Rett syndrome. Mellén et al. find the methyl-CpG-binding domain of MeCP2 interacts with hydroxymethyl-CpG.
منابع مشابه
Sequence‐specific DNA binding by AT‐hook motifs in MeCP2
MeCP2 is a chromatin-associated protein that is mutated in Rett syndrome. Its methyl-CpG-binding domain interacts with DNA containing methylated cytosine, but other modes of recruitment to the genome have also been proposed. Here, we use in vitro and in vivo assays to investigate the DNA binding specificity of two AT-hook motifs in MeCP2. One exhibits robust sequence-specific DNA binding, where...
متن کاملA novel DNA-binding feature of MeCP2 contributes to Rett syndrome
Rett syndrome (RTT) is an X-linked neurodevelopmental disorder associated with intellectual disabilities, which almost exclusively affects females during early childhood with an incidence of 1:10,000– 15,000 worldwide (Neul and Zoghbi, 2004). RTT is primarily caused by lossof-function mutations in methyl-CpGbinding protein 2 (MECP2) (Amir et al., 1999), the gene encoding MeCP2, a transcriptiona...
متن کاملAn AT-Hook Domain in MeCP2 Determines the Clinical Course of Rett Syndrome and Related Disorders
Mutations in the X-linked MECP2 cause Rett syndrome, a devastating neurological disorder typified by a period of apparently normal development followed by loss of cognitive and psychomotor skills. Data from rare male patients suggest symptom onset and severity can be influenced by the location of the mutation, with amino acids 270 and 273 marking the difference between neonatal encephalopathy a...
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Background Mutations in Methyl-CpG binding protein 2 (MECP2) have been identified as the disease-causing mutations in Rett Syndrome (RTT). However, no mutation in the AT-hook 1 domain of MECP2 has been reported in RTT yet. The function of AT-hook 1 domain of MECP2 has not been described either. Methods The clinical and radiological features of a girl with progressive hyperactivity and loss of...
متن کاملComparative analysis of linker histone H1, MeCP2, and HMGD1 on nucleosome stability and target site accessibility
Chromatin architectural proteins (CAPs) bind the entry/exit DNA of nucleosomes and linker DNA to form higher order chromatin structures with distinct transcriptional outcomes. How CAPs mediate nucleosome dynamics is not well understood. We hypothesize that CAPs regulate DNA target site accessibility through alteration of the rate of spontaneous dissociation of DNA from nucleosomes. We investiga...
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ورودعنوان ژورنال:
- Cell
دوره 152 شماره
صفحات -
تاریخ انتشار 2013