Hes1 expression is reduced in Tbx1 null cells and is required for the development of structures affected in 22q11 deletion syndrome.

Journal article

22q11 deletion syndrome (22q11DS) is characterised by aberrant development of the pharyngeal apparatus and the heart with haploinsufficiency of the transcription factor TBX1 being considered the major underlying cause of the disease. Tbx1 mutations in mouse phenocopy the disorder. In order to identify the transcriptional dysregulation in Tbx1-expressing lineages we optimised fluorescent-activated cell sorting of beta-galactosidase expressing cells (FACS-Gal) to compare the expression profile ...

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Authors: van Bueren, K; Papangeli, I; Rochais, F; Pearce, K; Roberts, C

• Publication status: Published
• Journal: Developmental biology
• Volume: 340
• Issue: 2
• Pages: 369-380
• Publication date: 2010-04-01
• DOI: 10.1016/j.ydbio.2010.01.020
• EISSN: 1095-564X
• ISSN: 0012-1606
• Language: English
• Keywords: beta-Galactosidase; Chromosomes; Basic Helix-Loop-Helix Transcription Factors; Heart; Thymus Gland; T-Box Domain Proteins; Mice, Knockout; Sequence Deletion; Syndrome; Branchial Region; In Situ Hybridization; Animals; Embryo, Mammalian; Homeodomain Proteins; Mice