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Suppressor of fused homolog

SUFU, Su(fu)
The Hedgehog signaling pathway plays an important role in early human development. The pathway is a signaling cascade that plays a role in pattern formation and cellular proliferation during development. This gene encodes a negative regulator of the hedgehog signaling pathway. Defects in this gene are a cause of medulloblastoma. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010] (from NCBI)
Top mentioned proteins: Gli, Smo, Shh, THP, CAN
Papers on SUFU
Targeting hedgehog signaling pathway in pediatric tumors: in vitro evaluation of SMO and GLI inhibitors.
Lanvers-Kaminsky et al., Berlin, Germany. In Cancer Chemother Pharmacol, Feb 2016
RESULTS: Key members of hedgehog signaling (SHH, PTCH1, SMO, GLI1, GLI2 and SUFU) were expressed in all cell lines.
Hedgehog signaling pathway: a novel model and molecular mechanisms of signal transduction.
Gorojankina, Gif-sur-Yvette, France. In Cell Mol Life Sci, Feb 2016
It also suggests a novel role for the negative pathway regulators Sufu and PKA in these processes.
Identification of genomic mutations associated with clinical outcomes of induction chemotherapy in patients with head and neck squamous cell carcinoma.
Heo et al., Seoul, South Korea. In J Cancer Res Clin Oncol, Jan 2016
Thirty-three mutations in TP53, NOTCH3, FGFR2, FGFR3, ATM, EGFR, MET, PTEN, FBXW7, SYNE1, and SUFU were frequently altered in poor responders.
Multiple Hereditary Infundibulocystic Basal Cell Carcinoma Syndrome Associated With a Germline SUFU Mutation.
Cho et al., San Francisco, United States. In Jama Dermatol, Jan 2016
A conserved splice-site mutation in 1 copy of the suppressor of fused gene (SUFU) was identified in all tumor and normal tissue samples.
Germline and somatic mutations in meningiomas.
Smith, Manchester, United Kingdom. In Cancer Genet, Apr 2015
The sonic hedgehog (SHH)-GLI1 signaling pathway gene, SUFU, has also been identified as the cause of hereditary multiple meningiomas in a large Finnish family.
Genomic analysis of smoothened inhibitor resistance in basal cell carcinoma.
de Sauvage et al., San Francisco, United States. In Cancer Cell, Apr 2015
Genomic analysis of tumor biopsies revealed that vismodegib resistance is associated with Hedgehog (Hh) pathway reactivation, predominantly through mutation of the drug target SMO and to a lesser extent through concurrent copy number changes in SUFU and GLI2.
Germline mutations in SUFU cause Gorlin syndrome-associated childhood medulloblastoma and redefine the risk associated with PTCH1 mutations.
Evans et al., Manchester, United Kingdom. In J Clin Oncol, 2015
RESULTS: A germline heterozygous nonsense mutation in SUFU was identified in one of four exomes.
[Identification of a Family with SUFU Germline Deletion Based on a Case of Desmoplastic Medulloblastoma in an Infant].
Zitterbart et al., In Klin Onkol, 2014
Further molecular analysis identified four basic subgroups of medulloblastoma: WNT, SHH, Group 3, and Group 4. The subgroup of SHH meduloblastoma is associated with somatic mutations of SHH, PTCH1, SUFU, SMO and TP53, while the most common mutations found in infants up to three years of age were PTCH1 and SUFU.
Epigenetic targeting of Hedgehog pathway transcriptional output through BET bromodomain inhibition.
Cho et al., Stanford, United States. In Nat Med, 2014
We show that BRD4 and other BET bromodomain proteins regulate GLI transcription downstream of SMO and suppressor of fused (SUFU), and chromatin immunoprecipitation studies reveal that BRD4 directly occupies GLI1 and GLI2 promoters, with a substantial decrease in engagement of these sites after treatment with JQ1, a small-molecule inhibitor targeting BRD4.
The kinesin-4 protein Kif7 regulates mammalian Hedgehog signalling by organizing the cilium tip compartment.
Anderson et al., New York City, United States. In Nat Cell Biol, 2014
Mammalian Hedgehog (Hh) signal transduction requires a primary cilium, a microtubule-based organelle, and the Gli-Sufu complexes that mediate Hh signalling, which are enriched at cilia tips.
Genome sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition.
ICGC PedBrain Tumor Project et al., Heidelberg, Germany. In Cancer Cell, 2014
SHH pathway mutations involved PTCH1 (across all age groups), SUFU (infants, including germline), and SMO (adults).
Small-molecule antagonists of Gli function
Chen et al., Bethesda, United States. In Unknown Journal, 2013
How Smo regulates Gli transcription factor function remains unclear, but this process involve the scaffolding protein Suppressor of Fused (Sufu), which can directly inhibit the Gli proteins and facilitate their proteolytic processing into N-terminal repressors.
The sonic hedgehog-GLI1 signaling pathway in brain tumor development.
Castresana et al., Davis, United States. In Expert Opin Ther Targets, 2012
Other proteins like HHIP, SUFU, Bmi-1, Cyclin D2, Plakoglobin, PAX6, Nkx2.2, and SFRP1 are not so well understood in Shh regulation as Gli-1 downstream target genes.
High frequency of germline SUFU mutations in children with desmoplastic/nodular medulloblastoma younger than 3 years of age.
Grill et al., Villejuif, France. In J Clin Oncol, 2012
germline SUFU mutations are responsible for a high proportion of desmoplastic medulloblastoma in children younger than 3 years of age
Rab23 negatively regulates Gli1 transcriptional factor in a Su(Fu)-dependent manner.
Xie et al., Indianapolis, United States. In Cell Signal, 2012
Rab23 directly associates with Su(Fu) and inhibits Gli1 function in a Su(Fu)-dependent manner.
Dual function of suppressor of fused in Hh pathway activation and mouse spinal cord patterning.
Liu et al., United States. In Dev Biol, 2012
Sufu plays a positive role in the maximal activation of Hh signaling that defines the ventral-most cell fate in the mammalian spinal cord, likely through protecting Gli2 and Gli3 proteins from degradation.
Novel mechanism of action on Hedgehog signaling by a suppressor of fused carboxy terminal variant.
Zaphiropoulos et al., Huddinge, Sweden. In Plos One, 2011
mechanism of action on Hedgehog signaling by a suppressor of fused carboxy terminal variant
Medulloblastomas in adults: prognostic factors and lessons from paediatrics.
Stupp et al., Bern, Switzerland. In Curr Opin Neurol, 2011
Activation of the sonic hedgehog pathway with frequent mutations of the PTCH and SUFU genes, loss of 9q, and positivity for GLI1 and SFRP1 is more frequent in children less than 3 years old and in adults, commonly associated with desmoplastic histology.
Kif7 promotes hedgehog signaling in growth plate chondrocytes by restricting the inhibitory function of Sufu.
Alman et al., Toronto, Canada. In Development, 2011
Kif7 regulates the activity of Gli transcription factors through both Sufu-dependent and -independent mechanisms.
Nevoid Basal Cell Carcinoma Syndrome
Farndon et al., Seattle, United States. In Unknown Journal, 2002
The risk of developing medulloblastoma is substantially higher in individuals with an SUFU pathogenic variant (33%) than in those with a PTCH1 pathogenic variant (<2%).
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