Gene Symbol: GLI3
Description: GLI family zinc finger 3
Alias: ACLS, GCPS, GLI3-190, GLI3FL, PAP-A, PAPA, PAPA1, PAPB, PHS, PPDIV, transcriptional activator GLI3, GLI-Kruppel family member GLI3, glioma-associated oncogene family zinc finger 3, oncogene GLI3, zinc finger protein GLI3
Species: human
Products:     GLI3

Top Publications

  1. Ruppert J, Kinzler K, Wong A, Bigner S, Kao F, Law M, et al. The GLI-Kruppel family of human genes. Mol Cell Biol. 1988;8:3104-13 pubmed
    ..The predicted proteins probably control the expression of other genes and, by analogy with Kr and GLI, may be important in human development, tissue-specific differentiation, or neoplasia. ..
  2. Wang Z, Wang J, Li Y, Geng J, Fu Q, Xu Y, et al. Novel frame-shift mutations of GLI3 gene in non-syndromic postaxial polydactyly patients. Clin Chim Acta. 2014;433:195-9 pubmed publisher
    ..The glioma-associated oncogene family zinc finger 3 (GLI3) is known to be associated with both syndromic and non-syndromic polydactyly...
  3. Dai P, Akimaru H, Tanaka Y, Maekawa T, Nakafuku M, Ishii S. Sonic Hedgehog-induced activation of the Gli1 promoter is mediated by GLI3. J Biol Chem. 1999;274:8143-52 pubmed
    ..Here, we report distinct roles for GLI1 and GLI3 in Sonic hedgehog (Shh) signaling...
  4. Naruse I, Ueta E, Sumino Y, Ogawa M, Ishikiriyama S. Birth defects caused by mutations in human GLI3 and mouse Gli3 genes. Congenit Anom (Kyoto). 2010;50:1-7 pubmed publisher
    ABSTRACT GLI3 is the gene responsible for Greig cephalopolysyndactyly syndrome (GCPS), Pallister-Hall syndrome (PHS) and Postaxial polydactyly type-A (PAP-A)...
  5. Endoh Yamagami S, Evangelista M, Wilson D, Wen X, Theunissen J, Phamluong K, et al. The mammalian Cos2 homolog Kif7 plays an essential role in modulating Hh signal transduction during development. Curr Biol. 2009;19:1320-6 pubmed publisher
    ..We also demonstrate a requirement for Kif7 in the efficient localization of Gli3 to cilia in response to Hh and for the processing of Gli3 to its repressor form...
  6. Abbasi A, Paparidis Z, Malik S, Goode D, Callaway H, Elgar G, et al. Human GLI3 intragenic conserved non-coding sequences are tissue-specific enhancers. PLoS ONE. 2007;2:e366 pubmed
    The zinc-finger transcription factor GLI3 is a key regulator of development, acting as a primary transducer of Sonic hedgehog (SHH) signaling in a combinatorial context dependent fashion controlling multiple patterning steps in different ..
  7. Pan Y, Wang B. A novel protein-processing domain in Gli2 and Gli3 differentially blocks complete protein degradation by the proteasome. J Biol Chem. 2007;282:10846-52 pubmed
    ..The molecular mechanism for such limited degradation is unknown. The repressor forms of Gli2 and Gli3 transcription factors are generated from their full-length proteins through limited proteasome-mediated protein ..
  8. Zhang X, Jin C, Liu L, Zhao N, Zhang L, Ji S, et al. [Association and mutation analysis of GLI3 gene in idiopathic congenital talipes equinovarus]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2006;23:551-4 pubmed
    To explore the association and mutation of GLI3 gene in idiopathic congenital talipes equinovarus(ICTEV)...
  9. Wang C, Ruther U, Wang B. The Shh-independent activator function of the full-length Gli3 protein and its role in vertebrate limb digit patterning. Dev Biol. 2007;305:460-9 pubmed
    ..A/P) limb patterning in vertebrates is determined by the counteraction between the Sonic Hedgehog (Shh) and the Gli3 transcription factor...

More Information


  1. Sethi S, Goyal D, Khalil S, Yadav D. Two Indian families with Greig cephalopolysyndactyly with non-syndromic phenotype. Eur J Pediatr. 2013;172:1131-5 pubmed publisher
    The Greig cephalopolysyndactyly syndrome (GCPS) is a rare, autosomal dominant, pleiotropic, multiple congenital anomaly syndrome. The typical findings include hypertelorism, macrocephaly with frontal bossing, and polysyndactyly...
  2. Liu H, Zhang J, Song S, Zhao H, Han D, Feng H. A case-control study of the association between tooth-development gene polymorphisms and non-syndromic hypodontia in the Chinese Han population. Eur J Oral Sci. 2012;120:378-85 pubmed publisher
    ..and controls, in the allele and genotype frequencies of two markers [rs929387 of GLI family zinc finger 3 (GLI3) and rs11001553 of Dickkopf-related protein 1 (DKK1)]...
  3. Johnston J, Sapp J, Turner J, Amor D, Aftimos S, Aleck K, et al. Molecular analysis expands the spectrum of phenotypes associated with GLI3 mutations. Hum Mutat. 2010;31:1142-54 pubmed publisher
    A range of phenotypes including Greig cephalopolysyndactyly and Pallister-Hall syndromes (GCPS, PHS) are caused by pathogenic mutation of the GLI3 gene...
  4. Haycraft C, Banizs B, Aydin Son Y, Zhang Q, Michaud E, Yoder B. Gli2 and Gli3 localize to cilia and require the intraflagellar transport protein polaris for processing and function. PLoS Genet. 2005;1:e53 pubmed
    ..Our data show that loss of Tg737 results in altered Gli3 processing that abrogates Gli3-mediated repression of Gli1 transcriptional activity...
  5. Elson E, Perveen R, Donnai D, Wall S, Black G. De novo GLI3 mutation in acrocallosal syndrome: broadening the phenotypic spectrum of GLI3 defects and overlap with murine models. J Med Genet. 2002;39:804-6 pubmed
    ..The condition overlaps with Greig cephalopolysyndactyly syndrome (GCPS), an autosomal dominant disorder that results from mutations in the GLI3 gene...
  6. Pavletich N, Pabo C. Crystal structure of a five-finger GLI-DNA complex: new perspectives on zinc fingers. Science. 1993;261:1701-7 pubmed
    ..Analyzing the GLI complex and comparing it with Zif268 offers new perspectives on Zn finger-DNA recognition. ..
  7. Kang S, Graham J, Olney A, Biesecker L. GLI3 frameshift mutations cause autosomal dominant Pallister-Hall syndrome. Nat Genet. 1997;15:266-8 pubmed
    ..Am. J. Hum. Genet. 59, A81 (1996)), co-localizing the PHS locus and the GLI3 zinc finger transcription factor gene...
  8. Villavicencio E, Walterhouse D, Iannaccone P. The sonic hedgehog-patched-gli pathway in human development and disease. Am J Hum Genet. 2000;67:1047-54 pubmed
  9. Radhakrishna U, Wild A, Grzeschik K, Antonarakis S. Mutation in GLI3 in postaxial polydactyly type A. Nat Genet. 1997;17:269-71 pubmed
  10. Murone M, Luoh S, Stone D, Li W, Gurney A, Armanini M, et al. Gli regulation by the opposing activities of fused and suppressor of fused. Nat Cell Biol. 2000;2:310-2 pubmed
  11. Kalff Suske M, Wild A, Topp J, Wessling M, Jacobsen E, Bornholdt D, et al. Point mutations throughout the GLI3 gene cause Greig cephalopolysyndactyly syndrome. Hum Mol Genet. 1999;8:1769-77 pubmed be associated with translocations as well as point mutations affecting one allele of the zinc finger gene GLI3. In addition to GCPS, Pallister-Hall syndrome (PHS; MIM 146510) and post-axial polydactyly type A (PAP-A; MIM ..
  12. Cheng S, Bishop J. Suppressor of Fused represses Gli-mediated transcription by recruiting the SAP18-mSin3 corepressor complex. Proc Natl Acad Sci U S A. 2002;99:5442-7 pubmed
    ..These results provide biochemical evidence that Su(fu) directly participates in modulating the transcriptional activity of Gli. ..
  13. Iwasaki H, Nakano K, Shinkai K, Kunisawa Y, Hirahashi M, Oda Y, et al. Hedgehog Gli3 activator signal augments tumorigenicity of colorectal cancer via upregulation of adherence-related genes. Cancer Sci. 2013;104:328-36 pubmed publisher
    Hedgehog signal is re-activated in several cancers. In this study, we examined the role of Gli3 on malignant phenotype of tumorigenicity for colorectal cancer and its relationship with p53, WNT and ERK/AKT signals...
  14. Wild A, Kalff Suske M, Vortkamp A, Bornholdt D, Konig R, Grzeschik K. Point mutations in human GLI3 cause Greig syndrome. Hum Mol Genet. 1997;6:1979-84 pubmed
    Greig cephalopolysyndactyly syndrome (GCPS, MIM 175700) is a rare autosomal dominant developmental disorder characterized by craniofacial abnormalities and post-axial and pre-axial polydactyly as well as syndactyly of hands and feet...
  15. Shin S, Kogerman P, Lindstrom E, Toftgard R, Biesecker L. GLI3 mutations in human disorders mimic Drosophila cubitus interruptus protein functions and localization. Proc Natl Acad Sci U S A. 1999;96:2880-4 pubmed
    Truncation mutations of the GLI3 zinc finger transcription factor can cause Greig cephalopolysyndactyly syndrome (GCPS), Pallister-Hall syndrome (PHS), and postaxial polydactyly type A (PAP-A)...
  16. Krauss S, Foerster J, Schneider R, Schweiger S. Protein phosphatase 2A and rapamycin regulate the nuclear localization and activity of the transcription factor GLI3. Cancer Res. 2008;68:4658-65 pubmed publisher
    ..Three SHH effectors, GLI1, GLI2, and GLI3, regulate transcription of diverse genes involved in cell growth and cell proliferation...
  17. Alvarez Medina R, Cayuso J, Okubo T, Takada S, Marti E. Wnt canonical pathway restricts graded Shh/Gli patterning activity through the regulation of Gli3 expression. Development. 2008;135:237-47 pubmed
    ..Additionally, we show that Gli3 expression is controlled by Wnt activity...
  18. Radhakrishna U, Bornholdt D, Scott H, Patel U, Rossier C, Engel H, et al. 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. Am J Hum Genet. 1999;65:645-55 pubmed
    ..Mutations in the human GLI3 gene have been identified in Greig cepalopolysyndactyly, Pallister-Hall syndrome (PHS), and postaxial polydactyly ..
  19. Zhou H, Kim S, Ishii S, Boyer T. Mediator modulates Gli3-dependent Sonic hedgehog signaling. Mol Cell Biol. 2006;26:8667-82 pubmed
    ..Here, we identify and characterize a physical and functional interaction between Gli3 and the MED12 subunit within the RNA polymerase II transcriptional Mediator...
  20. Tempé D, Casas M, Karaz S, Blanchet Tournier M, Concordet J. Multisite protein kinase A and glycogen synthase kinase 3beta phosphorylation leads to Gli3 ubiquitination by SCFbetaTrCP. Mol Cell Biol. 2006;26:4316-26 pubmed
    b>Gli3 is a zinc finger transcription factor proteolytically processed into a truncated repressor lacking C-terminal activation domains...
  21. Johnston J, Olivos Glander I, Killoran C, Elson E, Turner J, Peters K, et al. Molecular and clinical analyses of Greig cephalopolysyndactyly and Pallister-Hall syndromes: robust phenotype prediction from the type and position of GLI3 mutations. Am J Hum Genet. 2005;76:609-22 pubmed
    Mutations in the GLI3 zinc-finger transcription factor gene cause Greig cephalopolysyndactyly syndrome (GCPS) and Pallister-Hall syndrome (PHS), which are variable but distinct clinical entities...
  22. Dai P, Shinagawa T, Nomura T, Harada J, Kaul S, Wadhwa R, et al. Ski is involved in transcriptional regulation by the repressor and full-length forms of Gli3. Genes Dev. 2002;16:2843-8 pubmed
    Transcription factor Glioblastoma-3 (Gli3) is cleaved in the anterior region of the limb bud to generate its repressor form...
  23. Ruppert J, Vogelstein B, Arheden K, Kinzler K. GLI3 encodes a 190-kilodalton protein with multiple regions of GLI similarity. Mol Cell Biol. 1990;10:5408-15 pubmed
    ..A previously identified fragment of GLI3 genomic DNA was used to localize GLI3 to chromosome 7p13 and to isolate cDNA clones...
  24. Stone D, Murone M, Luoh S, Ye W, Armanini M, Gurney A, et al. Characterization of the human suppressor of fused, a negative regulator of the zinc-finger transcription factor Gli. J Cell Sci. 1999;112 ( Pt 23):4437-48 pubmed
    ..factor Gli, which mediates Hedgehog signaling in vertebrates, and to physically interact with Gli, Gli2 and Gli3 as well as with Slimb, an F-box containing protein which, in the fly, suppresses the Hedgehog response, in part by ..
  25. Wang B, Fallon J, Beachy P. Hedgehog-regulated processing of Gli3 produces an anterior/posterior repressor gradient in the developing vertebrate limb. Cell. 2000;100:423-34 pubmed
    ..We demonstrate that PKA-dependent processing of vertebrate Gli3 in developing limb similarly generates a potent repressor in a manner antagonized by apparent long-range signaling ..
  26. Tsanev R, Tiigimägi P, Michelson P, Metsis M, Østerlund T, Kogerman P. Identification of the gene transcription repressor domain of Gli3. FEBS Lett. 2009;583:224-8 pubmed publisher
    ..We have analyzed the sequences and identified a potential repressor domain in Gli2 and Gli3 and have tested this experimentally...
  27. Hurst J, Jenkins D, Vasudevan P, Kirchhoff M, Skovby F, Rieubland C, et al. Metopic and sagittal synostosis in Greig cephalopolysyndactyly syndrome: five cases with intragenic mutations or complete deletions of GLI3. Eur J Hum Genet. 2011;19:757-62 pubmed publisher
    Greig cephalopolysyndactyly syndrome (GCPS) is a multiple congenital malformation characterised by limb and craniofacial anomalies, caused by heterozygous mutation or deletion of GLI3...
  28. Kang H, Oh S, Kim J, Yoo Y. Abrogation of Gli3 expression suppresses the growth of colon cancer cells via activation of p53. Exp Cell Res. 2012;318:539-49 pubmed publisher
    ..Herein we investigated the critical regulation of Gli3-p53 in tumorigenesis of colon cancer cells and the molecular mechanisms underlying these effects...
  29. Garcia N, Bozzini N, Baiocchi G, da Cunha I, Maciel G, Soares Júnior J, et al. May Sonic Hedgehog proteins be markers for malignancy in uterine smooth muscle tumors?. Hum Pathol. 2016;50:43-50 pubmed publisher
    ..Immunohistochemical analysis showed that SMO, SUFU, GLI1, GLI3, and BMP4 expression gradually increased depending on to the histologic tissue type...
  30. Prasad A, Raju G, Sivalingam V, Girdhar A, Verma M, Vats A, et al. An acridine derivative, [4,5-bis{(N-carboxy methyl imidazolium)methyl}acridine] dibromide, shows anti-TDP-43 aggregation effect in ALS disease models. Sci Rep. 2016;6:39490 pubmed publisher
    ..Thus, AIM4 can be a lead molecule potentiating further therapeutic research for ALS. ..
  31. Saelao S, Maneerat S, Kaewsuwan S, Rabesona H, Choiset Y, Haertle T, et al. Inhibition of Staphylococcus aureus in vitro by bacteriocinogenic Lactococcus lactis KTH0-1S isolated from Thai fermented shrimp (Kung-som) and safety evaluation. Arch Microbiol. 2017;199:551-562 pubmed publisher
    ..The inhibitory effect of bacteriocin remained intact after treatment with different pHs and after heating, but was sensitive to some proteolytic enzymes...
  32. Mills M, Harami G, Seol Y, Gyimesi M, Martina M, Kovács Z, et al. RecQ helicase triggers a binding mode change in the SSB-DNA complex to efficiently initiate DNA unwinding. Nucleic Acids Res. 2017;45:11878-11890 pubmed publisher
    ..We propose that this bidirectional communication promotes efficient DNA processing and explains how SSB stimulates rather than inhibits RecQ activity. ..
  33. Pittman K, Cervantes P, Knoll L. Z-DNA Binding Protein Mediates Host Control of Toxoplasma gondii Infection. Infect Immun. 2016;84:3063-70 pubmed publisher
    ..Taken together, these results highlight a role for ZBP1 in assisting host control of T. gondii infection. ..
  34. Bonella F, Long X, Ohshimo S, Horimasu Y, Griese M, Guzman J, et al. MUC1 gene polymorphisms are associated with serum KL-6 levels and pulmonary dysfunction in pulmonary alveolar proteinosis. Orphanet J Rare Dis. 2016;11:48 pubmed publisher
    ..557, p?=?0.014). MUC1 rs4072037 A/A genotype is associated with more severe pulmonary dysfunction and a higher rate of disease progression in PAP patients. ..
  35. Campbell L, Raheem I, Malemud C, Askari A. The Relationship between NALP3 and Autoinflammatory Syndromes. Int J Mol Sci. 2016;17: pubmed publisher
    ..Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome is caused by mutations in the genes encoding proline-serine-threonine phosphatase interacting protein 1 (..
  36. Zonja B, Delgado A, Abad J, Perez S, Barcelo D. Abiotic amidine and guanidine hydrolysis of lamotrigine-N2-glucuronide and related compounds in wastewater: The role of pH and N2-substitution on reaction kinetics. Water Res. 2016;100:466-475 pubmed publisher
    ..that because of different imino tautomer equilibrium LMG (N2-H) and LMG-N2-oxide ((+)N2-O(-)) were stable at all pHs but N2-methyl-LMG (N2-CH3) as well as LMG-N2-G were susceptible to amidine and guanidine hydrolysis at basic pH...
  37. Maboloc E, Chan K. Resilience of the larval slipper limpet Crepidula onyx to direct and indirect-diet effects of ocean acidification. Sci Rep. 2017;7:12062 pubmed publisher
    ..7 and pH 7.3) for 14 days, and were fed with Isochrysis galbana cultured at these three respective pHs. pH, diet, nor their interactions had no effect on larval mortality...
  38. Wiebe M, Jamin A. The Barrier to Autointegration Factor: Interlocking Antiviral Defense with Genome Maintenance. J Virol. 2016;90:3806-3809 pubmed publisher
    ..In this gem, we discuss the unique position BAF occupies at the virus-host interface and how both viral and cellular mechanisms may regulate its capacity to act as a pro- or antiviral effector targeting viral DNA. ..
  39. Hu W, Yu X, Hu Q, Kong J, Li L, Zhang X. Methyl Orange removal by a novel PEI-AuNPs-hemin nanocomposite. J Environ Sci (China). 2017;53:278-283 pubmed publisher
    ..Adsorption and degradation experiments were carried out at different pHs, nanocomposite concentrations and UV irradiation times...
  40. Ishibashi Y, Oura S, Umemura K. Adsorption of DNA binding proteins to functionalized carbon nanotube surfaces with and without DNA wrapping. Eur Biophys J. 2017;46:541-547 pubmed publisher
    ..In addition, the DNA binding protein RecA was more adhesive than single-stranded DNA binding proteins to the functionalized SWNT surfaces. ..
  41. Kovacs G. Concepts and classification of neurodegenerative diseases. Handb Clin Neurol. 2017;145:301-307 pubmed publisher
    ..Translation of neuropathologic categories of neurodegenerative diseases into in vivo detectable biomarkers is only partly achieved but intensive research is performed to reach this goal. ..
  42. Pallebage Gamarallage M, Foxley S, Menke R, Huszar I, Jenkinson M, Tendler B, et al. Dissecting the pathobiology of altered MRI signal in amyotrophic lateral sclerosis: A post mortem whole brain sampling strategy for the integration of ultra-high-field MRI and quantitative neuropathology. BMC Neurosci. 2018;19:11 pubmed publisher
  43. Taglialegna A, Navarro S, Ventura S, Garnett J, Matthews S, Penadés J, et al. Staphylococcal Bap Proteins Build Amyloid Scaffold Biofilm Matrices in Response to Environmental Signals. PLoS Pathog. 2016;12:e1005711 pubmed publisher
    ..the N-terminus of the protein become aggregation-prone and self-assemble into amyloid-like structures under acidic pHs and low concentrations of calcium...
  44. Ennil Bektaş T. Reduction dye in paint and construction chemicals wastewater by improved coagulation-flocculation process. Water Sci Technol. 2017;76:2816-2820 pubmed publisher
    ..The experimental results showed that the treatment with all substances was very effective. The pHs of treated wastewater were obtained in the range of 5-7 without needing pH adjustment process...
  45. Mompeán M, Romano V, Pantoja Uceda D, Stuani C, Baralle F, Buratti E, et al. The TDP-43 N-terminal domain structure at high resolution. FEBS J. 2016;283:1242-60 pubmed publisher
    ..Structural data have been deposited in the Protein Data Bank under accession code: 2n4p. The NMR assignments have been deposited in the BMRB database under access code: 25675. ..
  46. Low J, Webb S, Silva A, Saathoff H, Ryan D, Torrado M, et al. CHD4 Is a Peripheral Component of the Nucleosome Remodeling and Deacetylase Complex. J Biol Chem. 2016;291:15853-66 pubmed publisher
    ..The addition of recombinant CHD4 to this nucleosome deacetylase complex reconstitutes a NuRD complex with nucleosome remodeling activity. These data contribute to our understanding of the architecture of the NuRD complex. ..
  47. Yin X, Shin H, Li J, Du G, Liu L, Chen J. Pgas, a Low-pH-Induced Promoter, as a Tool for Dynamic Control of Gene Expression for Metabolic Engineering of Aspergillus niger. Appl Environ Microbiol. 2017;83: pubmed publisher
    ..0 but functions efficiently at low pHs, such as pH 2.0...
  48. Carmona F, Gonzalez A, Sánchez M, Galvez N, Cuesta R, Capdevila M, et al. Varying iron release from transferrin and lactoferrin proteins. A laboratory experiment. Biochem Mol Biol Educ. 2017;45:521-527 pubmed publisher
    ..iron into the cell, while Lf competes with pathogens to sequester iron in biological fluids at more acidic pHs. ?During this experiment, the students will carry out iron loading and unloading on both human Lf and Tf and ..
  49. Perera N, Sheean R, Crouch P, White A, Horne M, Turner B. Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice. Hum Mol Genet. 2016;25:4080-4093 pubmed publisher
    ..Our data also reinforce a novel potential link between ALS and spinal bulbar muscular atrophy (SBMA), another motor neurodegenerative disease mediated by reduced AR function in motor neurons. ..
  50. Zhou Y, Chen L, Tao J, Shen J, Gong D, Yun R, et al. Effective cleavage of phosphodiester promoted by the zinc(II) and copper(II) inclusion complexes of β-cyclodextrin. J Inorg Biochem. 2016;163:176-184 pubmed publisher
    ..The kinetics of bis(4-nitrophenyl) phosphate (BNPP) hydrolysis catalyzed by G1, G2, 1 and 2 were examined at pHs ranging from 7.50 to 10.50 at 308±0.1K...
  51. Ikeda T, Sugie T, Shimizu A, Toi M. Patterns of clinical practice for sentinel lymph node biopsy in women with node-negative breast cancer: the results of a national survey in Japan. Breast Cancer. 2017;24:341-344 pubmed publisher
    ..early breast cancer for SLN biopsy while approximately 24 % received dye alone, which was especially prevalent in PHs. The fICG was used in only 12 % as a non-radioactive method but incentive package for fICG by national health ..
  52. Leblanc B, Moss T. In Vitro DNase I Footprinting. Methods Mol Biol. 2015;1334:17-27 pubmed publisher
    ..This technique can furthermore reveal if multiple sites for a DNA-binding protein are present on a same fragment and in such a case will also allow the comparison of their respective affinities. ..
  53. Kim E, Blair D. Function of the Histone-Like Protein H-NS in Motility of Escherichia coli: Multiple Regulatory Roles Rather than Direct Action at the Flagellar Motor. J Bacteriol. 2015;197:3110-20 pubmed publisher
    ..These findings call for a substantially revised interpretation of the literature concerning H-NS and flagellar motility and highlight the importance of H-NS in diverse regulatory processes involved in the motile-sessile transition. ..
  54. Wang Y, Cao W, Zhang X, Guo J. Abiotic nitrate loss and nitrogenous trace gas emission from Chinese acidic forest soils. Environ Sci Pollut Res Int. 2017;24:22679-22687 pubmed publisher
    ..of abiotic nitrate reduction in acidic forest soils based on sterilized anoxic soil incubations at different soil pHs and nitrate loadings. The results showed that 24.9, 53.4, and 88...
  55. Yabut O, Ng H, Fernandez G, Yoon K, Kuhn J, Pleasure S. Loss of Suppressor of Fused in Mid-Corticogenesis Leads to the Expansion of Intermediate Progenitors. J Dev Biol. 2016;4: pubmed publisher
    ..This correlates with abnormal levels of the repressor form of Gli3 (Gli3R) and the ectopic expression of Patched 1 (Ptch1), a Sonic Hedgehog (Shh) target gene...
  56. Cherry A, Finta C, Karlström M, Jin Q, Schwend T, Astorga Wells J, et al. Structural basis of SUFU-GLI interaction in human Hedgehog signalling regulation. Acta Crystallogr D Biol Crystallogr. 2013;69:2563-79 pubmed publisher
    ..These findings reveal the structure of the SUFU-GLI interface and suggest a mechanism for an essential regulatory step in Hedgehog signalling, offering possibilities for the development of novel pathway modulators and therapeutics. ..
  57. Ho Y, Li X, Jamison S, Harding H, McKinnon P, Ron D, et al. PERK Activation Promotes Medulloblastoma Tumorigenesis by Attenuating Premalignant Granule Cell Precursor Apoptosis. Am J Pathol. 2016;186:1939-1951 pubmed publisher
    ..It is believed that medulloblastomas in Ptch1(+/-) mice results from the transformation of granule cell precursors (GCPs) in the developing cerebellum...
  58. Wang Y, Banerjee S, Ding L, Cai C, Wei F, Cai Q. The regulatory role of protein phosphorylation in human gammaherpesvirus associated cancers. Virol Sin. 2017;32:357-368 pubmed publisher
  59. Lagunas A, Wu J, Crowe D. Telomere DNA damage signaling regulates cancer stem cell evolution, epithelial mesenchymal transition, and metastasis. Oncotarget. 2017;8:80139-80155 pubmed publisher
    ..Surprisingly we discovered that metastatic clones evolved prior to histopathologic onset of primary tumors. These results have important implications for understanding the evolution and treatment of metastatic cancer...
  60. Lee H, Seo S, Lee S, Jeong H, Woo S, Lee K, et al. [18F]-THK5351 PET Imaging in Patients With Semantic Variant Primary Progressive Aphasia. Alzheimer Dis Assoc Disord. 2017;: pubmed publisher
    ..Recently selective tau tracers for positron emission tomography (PET) have been developed to determine the presence of cerebral tau deposits in vivo. Here, we investigated the topographical distribution of THK5351 in svPPA patients...
  61. Tarui T, Kim A, Flake A, McClain L, Stratigis J, Fried I, et al. Amniotic fluid transcriptomics reflects novel disease mechanisms in fetuses with myelomeningocele. Am J Obstet Gynecol. 2017;217:587.e1-587.e10 pubmed publisher
    ..Known genes that were associated with myelomeningocele (PRICKLE2, GLI3, RAB23, HES1, FOLR1) and novel dysregulated genes were identified in association with neurodevelopment and neuronal ..
  62. Guadamuro L, Flórez A, Alegría A, Vazquez L, Mayo B. Characterization of four β-glucosidases acting on isoflavone-glycosides from Bifidobacterium pseudocatenulatum IPLA 36007. Food Res Int. 2017;100:522-528 pubmed publisher
    ..Differences in activity were recorded for all four β-glucosidases at different pHs and temperatures under otherwise similar assay conditions, suggesting they have complementary activities under ..
  63. Le Gallo M, Rudd M, Urick M, Hansen N, Merino M, Mutch D, et al. The FOXA2 transcription factor is frequently somatically mutated in uterine carcinosarcomas and carcinomas. Cancer. 2018;124:65-73 pubmed publisher
    ..Cancer 2018;124:65-73. © 2017 American Cancer Society. ..
  64. Goh C, Lee I, Sundaram J, George S, Yusoff P, Brush M, et al. Chronic oxidative stress promotes GADD34-mediated phosphorylation of the TAR DNA-binding protein TDP-43, a modification linked to neurodegeneration. J Biol Chem. 2018;293:163-176 pubmed publisher
  65. Brazill D, Meyer L, Hatton R, Brock D, Gomer R. ABC transporters required for endocytosis and endosomal pH regulation in Dictyostelium. J Cell Sci. 2001;114:3923-32 pubmed
    ..RtoA, although having a normal endocytosis rate, have a decreased exocytosis rate and endosomes with abnormally low pHs. RtoA levels vary during the cell cycle, causing a cell-cycle-dependent modulation of parameters such as cytosolic ..
  66. Phadke S, Coadou E, Anouti M. Catholyte Formulations for High-Energy Li-S Batteries. J Phys Chem Lett. 2017;:5907-5914 pubmed publisher
    ..we demonstrate the beneficial effect of original catholyte formulations containing redox active organyl disulfides (PhS2Ph) on the capacity utilization and retention as well as the efficiency in LiS batteries...
  67. Van De Velde K, Chandler P, Van Der Straeten D, Rohde A. Differential coupling of gibberellin responses by Rht-B1c suppressor alleles and Rht-B1b in wheat highlights a unique role for the DELLA N-terminus in dormancy. J Exp Bot. 2017;68:443-455 pubmed publisher
    ..Importantly, two semi-dwarf ovg alleles increased dormancy, which correlated with improved pre-harvest sprouting (PHS) resistance...
  68. Fujioka H, Ariga T, Horiuchi K, Otsu M, Igawa H, Kawashima K, et al. Molecular analysis of non-syndromic preaxial polydactyly: preaxial polydactyly type-IV and preaxial polydactyly type-I. Clin Genet. 2005;67:429-33 pubmed
    Human GLI3 gene mutations have been identified in several phenotypes of digital abnormality such as Greig cephalopolysyndactyly syndrome, Pallister-Hall syndrome, preaxial polydactyly type-IV (PPD-IV) and postaxial polydactyly...
  69. O Rourke M, Soo K, Behringer R, Hui C, Tam P. Twist plays an essential role in FGF and SHH signal transduction during mouse limb development. Dev Biol. 2002;248:143-56 pubmed
    ..This is accompanied by disruption of the expression of genes (Shh, Gli1, Gli2, Gli3, and Ptch) associated with SHH signalling in the limb bud mesenchyme, the down-regulation of Bmp4 in the apical ..
  70. Geilen K, Heilmann M, Hillmer S, Böhmer M. WRKY43 regulates polyunsaturated fatty acid content and seed germination under unfavourable growth conditions. Sci Rep. 2017;7:14235 pubmed publisher
    ..In summary, WRKY43 encodes for a novel positive regulator of ABA-dependent gene regulation and as a potent modulator of fatty acid desaturation and seed filling, which results in increased tolerance to abiotic stress. ..
  71. De Mori R, Romani M, D Arrigo S, Zaki M, Lorefice E, Tardivo S, et al. Hypomorphic Recessive Variants in SUFU Impair the Sonic Hedgehog Pathway and Cause Joubert Syndrome with Cranio-facial and Skeletal Defects. Am J Hum Genet. 2017;101:552-563 pubmed publisher
    ..of the mutated residues, with loss of the native enveloping movement of the binding site around its ligand GLI3. Functional studies on cellular models and fibroblasts showed that both variants significantly reduced SUFU ..
  72. Ljubkovic M, Shi Y, Cheng Q, Bosnjak Z, Jiang M. Cardiac mitochondrial ATP-sensitive potassium channel is activated by nitric oxide in vitro. FEBS Lett. 2007;581:4255-9 pubmed
    ..We found that the mitoK(ATP) was activated by exogenous NO donor S-nitroso-N-acetyl penicillamine or PAPA NONOate. This activation was inhibited by mitoK(ATP) blockers 5-hydroxydecanoate or glibenclamide...
  73. St Amour I, Turgeon A, Goupil C, Planel E, Hébert S. Co-occurrence of mixed proteinopathies in late-stage Huntington's disease. Acta Neuropathol. 2018;135:249-265 pubmed publisher
    ..Further studies will be needed to decipher the impact of these proteinopathies on clinical manifestation of HD. ..
  74. Oh S, Kim J, Jang J, Hwang I, Woo J, Lim Y, et al. Age is related to neurological outcome in patients with out-of-hospital cardiac arrest (OHCA) receiving therapeutic hypothermia (TH). Am J Emerg Med. 2018;36:243-247 pubmed publisher
    ..61, 95% CI 1.23-10.57, p=0.019), short time from collapsed to ACLS (per 1min) (OR 1.12, 95% CI 1.06-1.18, p<0.001) had good CPC at 6-month post-admission...
  75. Li J, Cui Y, Xu J, Wang Q, Yang X, Li Y, et al. Suppressor of Fused restraint of Hedgehog activity level is critical for osteogenic proliferation and differentiation during calvarial bone development. J Biol Chem. 2017;292:15814-15825 pubmed publisher
    ..Deletion of Sufu in CNC leads to a discernible decrease in the repressive Gli3 form and an increase in the full-length Gli2...
  76. Irwin D, Lleo A, Xie S, McMillan C, Wolk D, Lee E, et al. Ante mortem cerebrospinal fluid tau levels correlate with postmortem tau pathology in frontotemporal lobar degeneration. Ann Neurol. 2017;82:247-258 pubmed publisher
    ..Autopsy-confirmed samples are critical for FTLD biomarker development and validation. Ann Neurol 2017;82:247-258. ..
  77. Tencer A, Cox K, Di L, Bridgers J, Lyu J, Wang X, et al. Covalent Modifications of Histone H3K9 Promote Binding of CHD3. Cell Rep. 2017;21:455-466 pubmed publisher
    ..Collectively, our data suggest that the targeting of covalently modified H3K9 by CHD3 might be essential in diverse functions of NuRD. ..
  78. Min B, Chung K. New Insights into Transglutaminase 2 and Links to Neurodegenerative Diseases. BMB Rep. 2017;: pubmed
    ..In this review, the authors discuss recent advances in TG2 biochemistry, several mechanisms of molecular regulation and pleotropic signaling functions, and the presumed role of TG2 in the progression of many NDDs...