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Nat Neurosci May 1, 2016; 19 (5): 678-89.

Chd7 cooperates with Sox10 and regulates the onset of CNS myelination and remyelination.

He D , Marie C , Zhao C , Kim B , Wang J , Deng Y , Clavairoly A , Frah M , Wang H , He X , Hmidan H , Jones BV , Witte D , Zalc B , Zhou X , Choo DI , Martin DM , Parras C , Lu QR .

Mutations in CHD7, encoding ATP-dependent chromodomain helicase DNA-binding protein 7, in CHARGE syndrome lead to multiple congenital anomalies, including craniofacial malformations, neurological dysfunction and growth delay. Mechanisms underlying the CNS phenotypes remain poorly understood. We found that Chd7 is a direct transcriptional target of oligodendrogenesis-promoting factors Olig2 and Smarca4/Brg1 and is required for proper onset of CNS myelination and remyelination. Genome-occupancy analyses in mice, coupled with transcriptome profiling, revealed that Chd7 interacted with Sox10 and targeted the enhancers of key myelinogenic genes. These analyses identified previously unknown Chd7 targets, including bone formation regulators Osterix (also known as Sp7) and Creb3l2, which are also critical for oligodendrocyte maturation. Thus, Chd7 coordinates with Sox10 to regulate the initiation of myelinogenesis and acts as a molecular nexus of regulatory networks that account for the development of a seemingly diverse array of lineages, including oligodendrocytes and osteoblasts, pointing to previously uncharacterized Chd7 functions in white matter pathogenesis in CHARGE syndrome.

PubMed ID: 26928066
PMC ID: PMC4846514
Article link:

Genes referenced: cdkn1a cdkn2b cdknx chd7 cnp creb3l2 enpp2 ermn hes1 hes5.2 id2 id4 lamtor2 mag mbp nfya olig2 pcsk7 pgrmc2 plp1 smarca4 sox10 sp7 tcf7l2 vsig1

Article Images: [+] show captions
References [+] :
Amiel, Temporal bone anomaly proposed as a major criteria for diagnosis of CHARGE syndrome. 2001, Pubmed

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