Abstract PURPOSE/METHODS: Clinical and molecular risk factors in 142 patients <5 years with desmoplastic medulloblastoma (DMB) or extensive nodularity (MBEN) were investigated. Patients diagnosed between 1992 2020 treated radiation-sparing approaches, 131 intraventricular methotrexate. 14 metastatic disease received high-dose chemotherapy. DNA methylation profiles of 77 sonic hedgehog (S...

Abstract Purpose The Sonic Hedgehog (Shh) pathway has been associated with a protective role after injury to the central nervous system (CNS). We, therefore, investigated effects of intrathecal Shh-administration in subacute phase thoracic spinal cord (SCI) on secondary processes rats. Methods Twenty-one Wistar rats were subjected clip-contusion/compression SCI at T9. Animals randomized into th...

BACKGROUND AND PURPOSE For diagnosis of medulloblastoma, the updated World Health Organization classification now demands for genetic typing, defining more precisely tumor biology, therapy, and prognosis. We investigated potential associations between magnetic resonance imaging (MRI) parameters including apparent diffusion coefficient (ADC) neuropathologic features focusing on subtypes. METHODS...

The mechanism of Sonic Hedgehog (Shh) pathway activation in non-small cell lung cancer (NSCLC) is poorly described. Using an antibody against the Shh C-terminal domain, we found a small population of Shh-positive (Shh+) cells in NSCLC cells. The objective of this study was to characterize these Shh+ cells. Shh+ and Shh- cells were sorted by using Fluorescence Activated Cell Sorting (FACS) on 12...

TheHedgehog (Hh) signaling response is regulated by the interaction of three key components that include the sonic hedgehog (Shh) ligand, its receptor patched 1 (Ptch1) and the pathway activator smoothened (Smo). Under the prevailing model of Shh pathway activation, the binding of Shh to Ptch1 (the keyShh receptor) results in the release of Ptch1-mediated inhibition of Smo, leading to Smo activ...

A potently active multivalent form of the protein Sonic hedgehog (Shh) was produced by bioconjugation of a modified recombinant form of Shh to the linear polymers poly(acrylic acid) (pAAc) and hyaluronic acid (HyA) via a two-step reaction exploiting carboimiide and maleimide chemistry. Efficiency of the conjugation was approximately 75% even at stoichiometric ratios of 30 Shh molecules per line...

To explore mechanisms controlling sonic hedgehog (Shh) expression in human cancers, we investigated regulation of Shh by the transcription factor NF-kappaB. We identify putative NF-kappaB binding sites in the human Shh promoter region that specifically bind NF-kappaB complexes. Further, NF-kappaB activation by tumor necrosis factor alpha (TNF-alpha) or p65 overexpression stimulates Shh promoter...

This study addresses the role of Sonic hedgehog (Shh) in promoting the generation of oligodendrocytes in the mouse telencephalon. We show that in the forebrain, expression of the early oligodendrocyte markers Olig2, plp/dm20 and PDGFR(alpha) corresponds to regions of Shh expression. To directly test if Shh can induce the development of oligodendrocytes within the telencephalon, we use retrovira...

Anteroposterior polarity in the vertebrate limb is thought to be regulated in response to signals derived from a specialized region of distal posterior mesenchyme, the zone of polarizing activity. Sonic Hedgehog (Shh) is expressed in the zone of polarizing activity and appears to mediate the action of the zone of polarizing activity. Here we have manipulated Shh signal in the limb to assess whe...