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Human SUFUH Protein expressed in Escherichia coli (E. coli) - ABIN667565
Dunaeva, Michelson, Kogerman, Toftgard: Characterization of the physical interaction of Gli proteins with SUFU proteins. in The Journal of biological chemistry 2003
Show all 2 references for ABIN667565
A Comparison of Ci/Gli (show GLI1 Proteins) Activity as Regulated by Sufu in Drosophila and Mammalian Hedgehog (show SHH Proteins) Response
Sufu can sequester Ci/Gli (show GLI1 Proteins) in the cytoplasm through binding to an N-terminal site while inhibiting Ci/Gli (show GLI1 Proteins) activity in the nucleus depending on a C-terminal Sufu-interacting site.
Drosophila Hedgehog (show SHH Proteins) signaling promotes downregulation of Su(fu) through its target protein HIB (Hh-induced BTB protein).
Suppressor of fused (Sufu) opposes Trn-mediated Ci nuclear import by masking its PY-nuclear localization sequence.
The 'closed' form of Sufu is stabilized by Gli (show GLI1 Proteins) binding and inhibited by Hh treatment, whereas the 'open' state of Sufu is promoted by Gli (show GLI1 Proteins)-dissociation and Hh signaling.
Su(fu) causes the Rdx (show RDX Proteins) switch between two mchanisms of Cubitus interruptus regulation.
Suppressor of Fused represses Gli (show GLI1 Proteins)-mediated transcription by recruiting the SAP18 (show SAP18 Proteins)-mSin3 corepressor complex.
Differential Hh target gene regulation can be accomplished by differential sensitivity of Cos2 and Su(Fu) to Hh.
Su(fu) protein levels and isoforms are crucial for the modulation of the different Ci states that control Hh target gene expression.
Sufu may act as a common regulator of Hh and Wnt (show WNT2 Proteins) signaling and contribute to intertwining the two pathways.
This study uncovers a previously unappreciated miR (show MLXIP Proteins)-214-Sufu pathway in controlling EMT (show ITK Proteins) and metastasis of lung adenocarcinoma.
A germline SUFU mutation was present in a patient with MHIBCC, and additional acquired SUFU mutations underlie the development of infundibulocystic basal cell carcinomas
SUFU germline polymorphism is associated with acute GVHD
Sufu has a role in repressing Gli1 (show GLI1 Proteins) transcription and nuclear accumulation, inhibiting glioma cell proliferation, invasion and vasculogenic mimicry, improving glioma chemo-sensitivity and prognosis
suggest childhood brain magnetic resonance imaging surveillance is justified in SUFU-related, but not PTCH1 (show PTCH1 Proteins)-related, Gorlin syndrome
SUFU polymorphisms are associated with neural tube defects in a high-risk population in China.
Functionally, RIOK3 (show RIOK3 Proteins) acts as a SUFU-dependent positive regulator of Hedgehog (show SHH Proteins) signaling.
Data indicate a significant role of hedgehog (show SHH Proteins) receptor PTCH1 (show PTCH1 Proteins) and SUFU in the pathogenesis of keratocystic odontogenic tumor (KCOT).
It is demonstrated that GLI (show GLI1 Proteins) binding is associated with major conformational changes in SUFU, including an intrinsically disordered loop that is also crucial for pathway activation.
Through regulation of su(fu), miR (show MYLIP Proteins)-214 enables precise specification of muscle cell types by sharpening cellular responses to Hedgehog (show SHH Proteins).
Sufu deletion early in embryogenesis resulted in unstable Gli2 and Gli3 (show GLI3 Proteins) activity, leading to the ectopic activation of Shh (show SHH Proteins) signaling.
Thus, we provide evidence that Sufu is involved in the genetic network that restricts the posterior expression of Gli2/3/Hand2 (show HAND2 Proteins) and Gremlin (show GREM1 Proteins)/Fgf in limb bud patterning.
Results showed that the Thr396 residue of Sufu is specifically required for regulation of Gli3 (show GLI3 Proteins) but not Gli2 implying a novel Sufu-mediated mechanism in which Gli2 activator and Gli3 (show GLI3 Proteins) repressor are differentially regulated.
findings demonstrate that perturbations of Sufu and Kif7 affect Gli (show GLI1 Proteins) activity and recapitulate the full spectrum of vertebrate limb defects, ranging from severe truncation to polydactyly.
Hh signaling results in reduced Sufu protein levels and Sufu dissociation from Gli (show GLI1 Proteins) proteins in the nucleus, highlighting critical functions of Sufu in the nucleus.
Data suggest nuclear entry of GLI1 (glioma-associated oncogene homolog (show GLI1 Proteins), a zinc finger protein) is regulated by unique mechanism via mutually exclusive binding by its nuclear import factor IMB1 (show KPNB1 Proteins) (importin (show KPNA4 Proteins) B1) and SuFu (suppressor of fused protein).
Differential regulation of Gli (show GLI1 Proteins) proteins by Sufu in the lung affects PDGF (show PDGFA Proteins) signaling and myofibroblast development.
Evc (show EVC Proteins) silencing in Sufu(-/-) cells attenuated the output of the Hh pathway, suggesting that Evc (show EVC Proteins)/Evc2 (show EVC2 Proteins) also promote Hh signaling in the absence of Sufu
Sufu acts universally as a negative regulator of Hedgehog (show SHH Proteins) signaling.
These studies establish Sufu and Kif7 as crucial components in the regulation of Gli2 localization and activity, and illustrate their overlapping functions in skin development and tumor suppression.
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.
, suppressor of fused
, suppressor of fused homolog