Targeted therapy done right: Direct sonic hedgehog inhibition for sonic hedgehog medulloblastoma. [PDF]
Nellan A, Jackson S.
europepmc +3 more sources
Atrioventricular canal defect and genetic syndromes: the unifying role of sonic hedgehog [PDF]
The atrioventricular canal defect (AVCD) is a congenital heart defect (CHD) frequently associated with extracardiac anomalies (75%). Previous observations from a personal series of patients with AVCD and "polydactyly syndromes" showed that the distinct ...
Baban, A +9 more
core +1 more source
A Notch above Sonic Hedgehog [PDF]
Cell fate specification requires tightly orchestrated cellular responses to extracellular factors. In this issue of Developmental Cell, Kong et al. (2015) reveal that Notch signaling activity facilitates the interpretation of Sonic hedgehog (Shh) signals by regulating signaling in the cilia, influencing fate specification of neural stem and progenitor ...
Yabut, Odessa +2 more
openaire +2 more sources
The Hedgehog (Hh) signal transduction pathway has been discovered as a central regulator of embryonic development, tissue maintenance and repair [1]. Moreover, several recent evidences have highlighted its key function in tumorigenesis [2]. In some familial cancers, such as basal cell carcinoma and medulloblastoma, Hh pathway activation represents the ...
Laukkanen M, Gutkind J, Castellone M
openaire +5 more sources
Dicer Is Required for Normal Cerebellar Development and to Restrain Medulloblastoma Formation. [PDF]
Dicer, a ribonuclease III enzyme, is required for the maturation of microRNAs. To assess its role in cerebellar and medulloblastoma development, we genetically deleted Dicer in Nestin-positive neural progenitors and in mice lacking one copy for the Sonic
Frederique Zindy +7 more
doaj +1 more source
Shortened primary cilium length and dysregulated Sonic hedgehog signaling in Niemann-Pick C1 disease [PDF]
The Niemann-Pick type C1 (NPC1) disease is a neurodegenerative lysosomal storage disorder due to mutations in the NPC1 gene, encoding a transmembrane protein related to the Sonic hedgehog receptor, Patched, and involved in intracellular trafficking of ...
Canterini, Sonia +7 more
core +1 more source
Sonic Hedgehog Signaling and VACTERL Association [PDF]
Hedgehog (Hh) signaling is vital for the patterning and organogenesis of almost every system. The specificity of these developmental processes is achieved through a tight spatio-temporal regulation of Hh signaling. Mice with defective Hh signal exhibit a wide spectrum of anomalies, including Vertebral defects, Anal atresia, Cardiovascular anomalies ...
Kim, KH, Hui, CC, Ngan, ESW
openaire +3 more sources
ObjectivesTo investigate the pathogenic effect of Porphyromonas gingivalis cultured media on the esophagus and the mechanism underlying the effect.BackgroundPeriodontitis is strongly associated with esophageal squamous cell carcinoma (ESCC). The cultured
Xueting Jia +7 more
doaj +1 more source
Regulation of sonic hedgehog-GLI1 downstream target genes PTCH1, Cyclin D2, Plakoglobin, PAX6 and NKX2.2 and their epigenetic status in medulloblastoma and astrocytoma [PDF]
Background The Sonic hedgehog (Shh) signaling pathway is critical for cell growth and differentiation. Impairment of this pathway can result in both birth defects and cancer.
Afzal, Mohammad +6 more
core +5 more sources
TULP4, a novel E3 ligase gene, participates in neuronal migration as a candidate in schizophrenia
Mutations identified from four SCZ pedigrees resulted in decreased TULP4 expression. Tulp4 knockdown caused delayed neuron migration in embryonic mice, and impaired cognition and prepulse inhibition in adult mice. These phenotypes may be related to TULP4 through its involvement in the formation of a novel E3 ubiquitin ligases.
Yan Bi +19 more
wiley +1 more source

