(Q38051564)

27 August 2017

title

Toward a systems-level understanding of the Hedgehog signaling pathway: defining the complex, robust, and fragile (English)

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27 August 2017

Pamela V Tran

1

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27 August 2017

Salil A Lachke

series ordinal

2

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27 August 2017

Rolf W Stottmann

series ordinal

3

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27 August 2017

publication date

11 October 2012

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Wiley interdisciplinary reviews. Systems biology and medicine

27 August 2017

published in

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27 August 2017

volume

5

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27 August 2017

issue

1

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27 August 2017

page(s)

83-100

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Outline of a concept for organismic systems biology

27 August 2017

cites work

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Systems biology: a brief overview

21 January 2018

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From systems biology to systems biomedicine

21 January 2018

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Systems pathology: a critical review

21 January 2018

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Towards a theory of biological robustness

21 January 2018

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Mutations affecting segment number and polarity in Drosophila

21 January 2018

1 reference

Pattern formation in the vertebrate neural tube: a sonic hedgehog morphogen-regulated transcriptional network

21 January 2018

1 reference

Mechanisms and functions of Hedgehog signalling across the metazoa

21 January 2018

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A novel protein-processing domain in Gli2 and Gli3 differentially blocks complete protein degradation by the proteasome

21 January 2018

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Mice deficient in the fused homolog do not exhibit phenotypes indicative of perturbed hedgehog signaling during embryonic development

21 January 2018

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The kinesin protein Kif7 is a critical regulator of Gli transcription factors in mammalian hedgehog signaling

21 January 2018

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Mouse Kif7/Costal2 is a cilia-associated protein that regulates Sonic hedgehog signaling

21 January 2018

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Kif7 promotes hedgehog signaling in growth plate chondrocytes by restricting the inhibitory function of Sufu.

21 January 2018

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Cilia and developmental signaling

21 January 2018

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A physiological view of the primary cilium

21 January 2018

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Ciliogenesis in normal human kidney development and post-natal life

21 January 2018

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Hedgehog signalling in the mouse requires intraflagellar transport proteins

21 January 2018

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Cilia and Hedgehog responsiveness in the mouse

21 January 2018

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Mouse intraflagellar transport proteins regulate both the activator and repressor functions of Gli transcription factors

21 January 2018

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Loss of the retrograde motor for IFT disrupts localization of Smo to cilia and prevents the expression of both activator and repressor functions of Gli

21 January 2018

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THM1 negatively modulates mouse sonic hedgehog signal transduction and affects retrograde intraflagellar transport in cilia

21 January 2018

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Intraflagellar transport protein 122 antagonizes Sonic Hedgehog signaling and controls ciliary localization of pathway components

21 January 2018

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Patched1 regulates hedgehog signaling at the primary cilium

21 January 2018

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Hedgehog signal transduction by Smoothened: pharmacologic evidence for a 2-step activation process

21 January 2018

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Gli2 trafficking links Hedgehog-dependent activation of Smoothened in the primary cilium to transcriptional activation in the nucleus

21 January 2018

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The output of Hedgehog signaling is controlled by the dynamic association between Suppressor of Fused and the Gli proteins

21 January 2018

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A mechanism for vertebrate Hedgehog signaling: recruitment to cilia and dissociation of SuFu-Gli protein complexes

21 January 2018

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Kinetics of hedgehog-dependent full-length Gli3 accumulation in primary cilia and subsequent degradation

21 January 2018

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Complex interactions between genes controlling trafficking in primary cilia

21 January 2018

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Sequential phosphorylation of smoothened transduces graded hedgehog signaling.

21 January 2018

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Lateral transport of Smoothened from the plasma membrane to the membrane of the cilium

21 January 2018

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Protein kinase A acts at the basal body of the primary cilium to prevent Gli2 activation and ventralization of the mouse neural tube

21 January 2018

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Kinome siRNA screen identifies regulators of ciliogenesis and hedgehog signal transduction

21 January 2018

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Application of active and kinase-deficient kinome collection for identification of kinases regulating hedgehog signaling

21 January 2018

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Inhibition of p70S6K2 down-regulates Hedgehog/GLI pathway in non-small cell lung cancer cell lines

21 January 2018

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EGF-ERBB signalling: towards the systems level

21 January 2018

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Vertebrate Hedgehog signalling modulated by induction of a Hedgehog-binding protein

21 January 2018

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The cell surface membrane proteins Cdo and Boc are components and targets of the Hedgehog signaling pathway and feedback network in mice

21 January 2018

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Smoothened mutants reveal redundant roles for Shh and Ihh signaling including regulation of L/R symmetry by the mouse node

21 January 2018

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Genetic and biochemical definition of the Hedgehog receptor

21 January 2018

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Overlapping roles and collective requirement for the coreceptors GAS1, CDO, and BOC in SHH pathway function

21 January 2018

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All mouse ventral spinal cord patterning by hedgehog is Gli dependent and involves an activator function of Gli3

21 January 2018

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Phosphorylation of Gli2 by protein kinase A is required for Gli2 processing and degradation and the Sonic Hedgehog-regulated mouse development

21 January 2018

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Vertebrate Smoothened functions at the primary cilium

21 January 2018

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Gli2 and Gli3 localize to cilia and require the intraflagellar transport protein polaris for processing and function

21 January 2018

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Transition zone proteins and cilia dynamics

21 January 2018

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The graded response to Sonic Hedgehog depends on cilia architecture

21 January 2018

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Fused kinase is stabilized by Cdc37/Hsp90 and enhances Gli protein levels

21 January 2018

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Novobiocin and additional inhibitors of the Hsp90 C-terminal nucleotide-binding pocket

21 January 2018

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Building the developmental oculome: systems biology in vertebrate eye development and disease

21 January 2018

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Interactome networks and human disease

21 January 2018

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Violations of robustness trade-offs

21 January 2018

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Gli proteins in development and disease

21 January 2018

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Mutations in the human Sonic Hedgehog gene cause holoprosencephaly

21 January 2018

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Identification of Sonic hedgehog as a candidate gene responsible for holoprosencephaly

21 January 2018

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The mutational spectrum of the sonic hedgehog gene in holoprosencephaly: SHH mutations cause a significant proportion of autosomal dominant holoprosencephaly.

21 January 2018

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How a Hedgehog might see holoprosencephaly

21 January 2018

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Maternally inherited heterozygous sequence change in the sonic hedgehog gene in a male patient with bilateral closed-lip schizencephaly and partial absence of the corpus callosum.

21 January 2018

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Novel mutation in sonic hedgehog in non-syndromic colobomatous microphthalmia.

21 January 2018

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Mutations in CDON, encoding a hedgehog receptor, result in holoprosencephaly and defective interactions with other hedgehog receptors

21 January 2018

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Mutations in IHH, encoding Indian hedgehog, cause brachydactyly type A-1

21 January 2018

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Homozygous mutations in IHH cause acrocapitofemoral dysplasia, an autosomal recessive disorder with cone-shaped epiphyses in hands and hips

21 January 2018

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A novel mutation of desert hedgehog in a patient with 46,XY partial gonadal dysgenesis accompanied by minifascicular neuropathy

21 January 2018

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Mutations in the desert hedgehog (DHH) gene in patients with 46,XY complete pure gonadal dysgenesis

21 January 2018

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Loss-of-function mutations in the human GLI2 gene are associated with pituitary anomalies and holoprosencephaly-like features

21 January 2018

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GLI2 mutations in four Brazilian patients: how wide is the phenotypic spectrum?

21 January 2018

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GLI3 frameshift mutations cause autosomal dominant Pallister-Hall syndrome

21 January 2018

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The phenotypic spectrum of GLI3 morphopathies includes autosomal dominant preaxial polydactyly type-IV and postaxial polydactyly type-A/B; No phenotype prediction from the position of GLI3 mutations

21 January 2018

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DYNC2H1 mutations cause asphyxiating thoracic dystrophy and short rib-polydactyly syndrome, type III

21 January 2018

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Ciliary abnormalities due to defects in the retrograde transport protein DYNC2H1 in short-rib polydactyly syndrome

21 January 2018

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KIF7 mutations cause fetal hydrolethalus and acrocallosal syndromes

21 January 2018

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Mutations in KIF7 link Joubert syndrome with Sonic Hedgehog signaling and microtubule dynamics

21 January 2018

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TTC21B contributes both causal and modifying alleles across the ciliopathy spectrum

21 January 2018

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Differential role of Rab proteins in ciliary trafficking: Rab23 regulates smoothened levels

21 January 2018

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RAB23 mutations in Carpenter syndrome imply an unexpected role for hedgehog signaling in cranial-suture development and obesity

21 January 2018

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Exome sequencing identifies WDR35 variants involved in Sensenbrenner syndrome

21 January 2018

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Human and mouse mutations in WDR35 cause short-rib polydactyly syndromes due to abnormal ciliogenesis

21 January 2018

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Mutations in the cilia gene ARL13B lead to the classical form of Joubert syndrome

21 January 2018

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A transition zone complex regulates mammalian ciliogenesis and ciliary membrane composition

21 January 2018

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A TCTN2 mutation defines a novel Meckel Gruber syndrome locus.

21 January 2018

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Somatic mutations in the human homologue of Drosophila patched in primitive neuroectodermal tumours

21 January 2018

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Human homolog of patched, a candidate gene for the basal cell nevus syndrome

21 January 2018

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Mutations of the human homolog of Drosophila patched in the nevoid basal cell carcinoma syndrome

21 January 2018

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The role of the human homologue of Drosophila patched in sporadic basal cell carcinomas

21 January 2018

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Identification of mutations in the human PATCHED gene in sporadic basal cell carcinomas and in patients with the basal cell nevus syndrome

21 January 2018

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Isolation and characterization of human patched 2 (PTCH2), a putative tumour suppressor gene inbasal cell carcinoma and medulloblastoma on chromosome 1p32.

21 January 2018

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Activating Smoothened mutations in sporadic basal-cell carcinoma

21 January 2018

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A frequent activated smoothened mutation in sporadic basal cell carcinomas

21 January 2018

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Mutations in SUFU predispose to medulloblastoma

21 January 2018

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Somatic mutations in the PTCH, SMOH, SUFUH and TP53 genes in sporadic basal cell carcinomas

21 January 2018

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Biological background of pediatric medulloblastoma and ependymoma: a review from a translational research perspective

21 January 2018

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Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer

21 January 2018

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Germline mutation of INI1/SMARCB1 in familial schwannomatosis

21 January 2018

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Basal cell carcinomas arise from hair follicle stem cells in Ptch1(+/-) mice

21 January 2018

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Hedgehog signalling within airway epithelial progenitors and in small-cell lung cancer

21 January 2018

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Hedgehog signalling in prostate regeneration, neoplasia and metastasis

21 January 2018

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Widespread requirement for Hedgehog ligand stimulation in growth of digestive tract tumours

21 January 2018

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Dual and opposing roles of primary cilia in medulloblastoma development

21 January 2018

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Primary cilia can both mediate and suppress Hedgehog pathway-dependent tumorigenesis

21 January 2018

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Two critical periods of Sonic Hedgehog signaling required for the specification of motor neuron identity

21 January 2018

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Suppression of the Shh pathway using a small molecule inhibitor eliminates medulloblastoma in Ptc1(+/-)p53(-/-) mice.

21 January 2018

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Discovery of NVP-LDE225, a Potent and Selective Smoothened Antagonist

21 January 2018

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Effective targeting of Hedgehog signaling in a medulloblastoma model with PF-5274857, a potent and selective Smoothened antagonist that penetrates the blood-brain barrier

21 January 2018

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In vitro and in vivo characterization of a novel Hedgehog signaling antagonist in human glioblastoma cell lines.

21 January 2018

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Identification of MK-5710 ((8aS)-8a-methyl-1,3-dioxo-2-[(1S,2R)-2-phenylcyclo- propyl]-N-(1-phenyl-1H-pyrazol-5-yl)hexahydro-imidazo[1,5-a]pyrazine-7(1H)-carboxamide), a potent smoothened antagonist for use in Hedgehog pathway dependent malignancies

21 January 2018

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A novel synthetic smoothened antagonist transiently inhibits pancreatic adenocarcinoma xenografts in a mouse model

21 January 2018

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Synthesis and biological evaluation of desmethylveramiline, a micromolar Hedgehog inhibitor

21 January 2018

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Identification and mechanism of action of the acylguanidine MRT-83, a novel potent Smoothened antagonist

21 January 2018

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Small molecule modulation of Smoothened activity

21 January 2018

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Virtual screening-based discovery and mechanistic characterization of the acylthiourea MRT-10 family as smoothened antagonists.

21 January 2018

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Discovery of a potent and orally active hedgehog pathway antagonist (IPI-926)

21 January 2018

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Inhibition of Hedgehog signaling enhances delivery of chemotherapy in a mouse model of pancreatic cancer

21 January 2018

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Converse conformational control of smoothened activity by structurally related small molecules

21 January 2018

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An orally bioavailable small-molecule inhibitor of Hedgehog signaling inhibits tumor initiation and metastasis in pancreatic cancer.

21 January 2018

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Small-molecule modulators of Hedgehog signaling: identification and characterization of Smoothened agonists and antagonists

21 January 2018

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Itraconazole, a commonly used antifungal that inhibits Hedgehog pathway activity and cancer growth

21 January 2018

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Inhibition of GLI-mediated transcription and tumor cell growth by small-molecule antagonists

21 January 2018

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Small-molecule inhibitors reveal multiple strategies for Hedgehog pathway blockade

21 January 2018

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Naturally occurring small-molecule inhibitors of hedgehog/GLI-mediated transcription

21 January 2018

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Common botanical compounds inhibit the hedgehog signaling pathway in prostate cancer

21 January 2018

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Oxysterols stimulate Sonic hedgehog signal transduction and proliferation of medulloblastoma cells

21 January 2018

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Purmorphamine activates the Hedgehog pathway by targeting Smoothened.

21 January 2018

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The Hedgehog's tale: developing strategies for targeting cancer

21 January 2018

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Molecular pathways: the role of primary cilia in cancer progression and therapeutics with a focus on Hedgehog signaling

21 January 2018

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Primary cilium depletion typifies cutaneous melanoma in situ and malignant melanoma

21 January 2018

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Primary cilia are decreased in breast cancer: analysis of a collection of human breast cancer cell lines and tissues

21 January 2018

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Pancreatic cancer and precursor pancreatic intraepithelial neoplasia lesions are devoid of primary cilia

21 January 2018

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A quantification of pathway components supports a novel model of Hedgehog signal transduction

21 January 2018

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Dynamic interpretation of hedgehog signaling in the Drosophila wing disc

21 January 2018

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The cis-regulatory logic of Hedgehog gradient responses: key roles for gli binding affinity, competition, and cooperativity

21 January 2018

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Cell-sorting at the A/P boundary in the Drosophila wing primordium: a computational model to consolidate observed non-local effects of Hh signaling

21 January 2018

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Isolation of primary cilia for morphological analysis

21 January 2018

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Proteomic analysis of mammalian primary cilia

21 January 2018

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Modeling the spatio-temporal network that drives patterning in the vertebrate central nervous system

21 January 2018

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A genome-scale analysis of the cis-regulatory circuitry underlying sonic hedgehog-mediated patterning of the mammalian limb

21 January 2018

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Hedgehog pathway-regulated gene networks in cerebellum development and tumorigenesis

21 January 2018

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A systems approach identifies HIPK2 as a key regulator of kidney fibrosis

21 January 2018

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21 January 2018

Identifiers

DOI

10.1002/WSBM.1193

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27 August 2017

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