Opening book details…
Can I read Interaction between PHOX2B and CREBBP mediates synergistic activation: Mechanistic implications of PHOX2B mutants on EtoBox?
Interaction between PHOX2B and CREBBP mediates synergistic activation: Mechanistic implications of PHOX2B mutants by Han-Tsang Wu; Yi-Ning Su; Chia-Cheng Hung; Wu-Shiun Hsieh; Kou-Juey Wu is a Biochemistry, Genetics and Molecular Biology article available to read on EtoBox.
What is Interaction between PHOX2B and CREBBP mediates synergistic activation: Mechanistic implications of PHOX2B mutants about?
Congenital central hypoventilation syndrome (CCHS) is a disorder of the autonomic nervous system. The genetic defect is usually caused by heterozygous mutations of the PHOX2B gene such as a 20-alanine tract (+5 to+13 alanines) expansion (approximately 95%) and occasional frameshift or missense mutations. Cytoplasmic aggregates were shown in PHOX2B proteins with longer alanine tract (+9 and longer) expansion and impaired DNA binding was observed in PHOX2B proteins with frameshift, missense, or longer alanine tract (+9 and longer) expansion. Defective transactivation activity was shown in certain PHOX2B mutants. However, PHOX2B proteins with short alanine tract (+5 to+7) expansion in the majority of patients (approximately 75%) did not have cytoplasmic aggregates or DNA binding defects. CREB-binding protein (CREBBP/CBP) is a transcriptional coactivator that interacts with multiple transcription factors to cause synergistic activation. Here we show that CBP interacted with PHOX2B and served as its coactivator to mediate synergistic activation. Wild-type PHOX2B and CBP used specific domains to interact with each other. The domains of CBP that interacted with different PHOX2B mutants we
Who reads Interaction between PHOX2B and CREBBP mediates synergistic activation: Mechanistic implications of PHOX2B mutants?
It is typically read by researchers, students, and practitioners in Biochemistry, Genetics and Molecular Biology.
- Author
- Han-Tsang Wu; Yi-Ning Su; Chia-Cheng Hung; Wu-Shiun Hsieh; Kou-Juey Wu
- Publisher
- Hindawi Limited
- Published
- 2009
- Language
- EN
- Field
- Biochemistry, Genetics and Molecular Biology (Life Sciences)