Experts and Doctors on high mobility group proteins in Houston, Texas, United States


Locale: Houston, Texas, United States
Topic: high mobility group proteins

Top Publications

  1. Su Z, Klein J, Du J, Franch H, Zhang L, Hassounah F, et al. Chronic kidney disease induces autophagy leading to dysfunction of mitochondria in skeletal muscle. Am J Physiol Renal Physiol. 2017;312:F1128-F1140 pubmed publisher
    ..However, myofibrillar protein is not directly broken down through autophagy. Instead, CKD-induced upregulation of autophagy leads to dysfunction of mitochondria and decrease of ATP production. ..
  2. Bien Willner G, Stankiewicz P, Lupski J. SOX9cre1, a cis-acting regulatory element located 1.1 Mb upstream of SOX9, mediates its enhancement through the SHH pathway. Hum Mol Genet. 2007;16:1143-56 pubmed
  3. Zhou G, Zheng Q, Engin F, Munivez E, Chen Y, Sebald E, et al. Dominance of SOX9 function over RUNX2 during skeletogenesis. Proc Natl Acad Sci U S A. 2006;103:19004-9 pubmed
    ..In summary, SOX9 is dominant over RUNX2 function in mesenchymal precursors that are destined for a chondrogenic lineage during endochondral ossification...
  4. Li L, Liu X, Glassman A, Keating M, Stros M, Plunkett W, et al. Fludarabine triphosphate inhibits nucleotide excision repair of cisplatin-induced DNA adducts in vitro. Cancer Res. 1997;57:1487-94 pubmed
    ..These results suggest that fludarabine may serve as a potential repair modulator to improve the antitumor efficacies of combination regimens containing agents that induce NER. ..
  5. Huang L, Li X, El Hodiri H, Dayal S, Wikramanayake A, Klein W. Involvement of Tcf/Lef in establishing cell types along the animal-vegetal axis of sea urchins. Dev Genes Evol. 2000;210:73-81 pubmed
    ..Our results suggest that SpTcf/Lef functions by interacting with beta-catenin to specify cell fates along the sea urchin animal-vegetal axis...
  6. Huang W, Chung U, Kronenberg H, de Crombrugghe B. The chondrogenic transcription factor Sox9 is a target of signaling by the parathyroid hormone-related peptide in the growth plate of endochondral bones. Proc Natl Acad Sci U S A. 2001;98:160-5 pubmed
  7. Zhao Q, Eberspaecher H, Lefebvre V, de Crombrugghe B. Parallel expression of Sox9 and Col2a1 in cells undergoing chondrogenesis. Dev Dyn. 1997;209:377-86 pubmed
  8. Patki G, Lau Y. Impact of exercise on mitochondrial transcription factor expression and damage in the striatum of a chronic mouse model of Parkinson's disease. Neurosci Lett. 2011;505:268-72 pubmed publisher
  9. Inoue K, Tanabe Y, Lupski J. Myelin deficiencies in both the central and the peripheral nervous systems associated with a SOX10 mutation. Ann Neurol. 1999;46:313-8 pubmed
    ..Our findings indicate that dysfunction of SOX10 may lead to deficiency of myelination in the central nervous system and peripheral nervous system as well as hypopigmentation and enteric aganglionosis. ..

More Information


  1. Stankiewicz P, Thiele H, Schlicker M, Cseke Friedrich A, Bartel Friedrich S, Yatsenko S, et al. Duplication of Xq26.2-q27.1, including SOX3, in a mother and daughter with short stature and dyslalia. Am J Med Genet A. 2005;138:11-7 pubmed
    ..Our findings further suggest that a dosage effect of SOX3 may to be responsible for a speech disorder in addition to short stature secondary to hypopituitarism...
  2. Hossain A, Saunders G. Role of Wilms tumor 1 (WT1) in the transcriptional regulation of the Mullerian-inhibiting substance promoter. Biol Reprod. 2003;69:1808-14 pubmed
    ..The WT1 is an essential factor for activation of the Mis promoter; therefore, the persistence of the mullerian duct in patients with Denys-Drash syndrome may result from deregulation of the MIS gene...
  3. Mori Akiyama Y, Akiyama H, Rowitch D, de Crombrugghe B. Sox9 is required for determination of the chondrogenic cell lineage in the cranial neural crest. Proc Natl Acad Sci U S A. 2003;100:9360-5 pubmed
    ..We hypothesize that these cells change their cell fate and acquire the ability to differentiate into osteoblasts. We conclude that Sox9 is required for the determination of the chondrogenic lineage in CNC cells. ..
  4. Inoue K, Khajavi M, Ohyama T, Hirabayashi S, Wilson J, Reggin J, et al. Molecular mechanism for distinct neurological phenotypes conveyed by allelic truncating mutations. Nat Genet. 2004;36:361-9 pubmed
    ..We observe similar results for truncating mutations of MPZ that convey distinct myelinopathies. Our experiments show that triggering NMD and escaping NMD may cause distinct neurological phenotypes. ..
  5. Qin Y, Kong L, Poirier C, Truong C, Overbeek P, Bishop C. Long-range activation of Sox9 in Odd Sex (Ods) mice. Hum Mol Genet. 2004;13:1213-8 pubmed
    ..We propose that Ods sex reversal is due to the Dct promoter element interacting with gonad-specific enhancer elements to produce the observed male pattern expression of Sox9 in the embryonic gonads. ..
  6. Zhang R, Murakami S, Coustry F, Wang Y, de Crombrugghe B. Constitutive activation of MKK6 in chondrocytes of transgenic mice inhibits proliferation and delays endochondral bone formation. Proc Natl Acad Sci U S A. 2006;103:365-70 pubmed
    ..Therefore, our study provides in vivo evidence for the role of p38 in endochondral ossification and suggests that Sox9 is a likely downstream target of the p38 MAPK pathway. ..
  7. Gao F, Maiti S, Alam N, Zhang Z, Deng J, Behringer R, et al. The Wilms tumor gene, Wt1, is required for Sox9 expression and maintenance of tubular architecture in the developing testis. Proc Natl Acad Sci U S A. 2006;103:11987-92 pubmed
    ..Our data, along with previous work demonstrating the role of Wt1 at early stages of gonadal development, thus indicate that Wt1 is essential at multiple steps in testicular development. ..
  8. Feng S, Bogatcheva N, Truong A, Korchin B, Bishop C, Klonisch T, et al. Developmental expression and gene regulation of insulin-like 3 receptor RXFP2 in mouse male reproductive organs. Biol Reprod. 2007;77:671-80 pubmed
    ..We determined the promoter region capable of providing the gubernacular-specific expression of Rxfp2. Analysis of RXFP2 promoter identified SOX9 as a new transcriptional enhancer of human gene expression. ..
  9. Huang W, Lu N, Eberspaecher H, de Crombrugghe B. A new long form of c-Maf cooperates with Sox9 to activate the type II collagen gene. J Biol Chem. 2002;277:50668-75 pubmed
    ..In summary, Lc-Maf is the first transcription factor shown to interact with Sox9, to be coexpressed with Sox9 during an early step of chondrogenesis and to cooperate with Sox9 in activating a downstream target gene of Sox9. ..
  10. Lefebvre V, de Crombrugghe B. Toward understanding SOX9 function in chondrocyte differentiation. Matrix Biol. 1998;16:529-40 pubmed
    ..Defining SOX9 function and the mechanisms that regulate SOX9 gene expression should contribute to a better understanding of chondrocyte differentiation. ..
  11. Lefebvre V, Li P, de Crombrugghe B. A new long form of Sox5 (L-Sox5), Sox6 and Sox9 are coexpressed in chondrogenesis and cooperatively activate the type II collagen gene. EMBO J. 1998;17:5718-33 pubmed
    ..Our data suggest that L-Sox5/Sox6 and Sox9, which belong to two different classes of Sox transcription factors, cooperate with each other in expression of Col2a1 and possibly other genes of the chondrocytic program. ..
  12. Wehrli B, Huang W, de Crombrugghe B, Ayala A, Czerniak B. Sox9, a master regulator of chondrogenesis, distinguishes mesenchymal chondrosarcoma from other small blue round cell tumors. Hum Pathol. 2003;34:263-9 pubmed
    ..More important, Sox9 may serve as a useful tool in the differentiation of small cell malignancies...
  13. Bi W, Huang W, Whitworth D, Deng J, Zhang Z, Behringer R, et al. Haploinsufficiency of Sox9 results in defective cartilage primordia and premature skeletal mineralization. Proc Natl Acad Sci U S A. 2001;98:6698-703 pubmed
    ..First, an early step presumably at the stage of mesenchymal condensation of cartilage primordia, and second, a later step preceding the transition of chondrocytes into hypertrophic chondrocytes...
  14. Huang W, Zhou X, Lefebvre V, de Crombrugghe B. Phosphorylation of SOX9 by cyclic AMP-dependent protein kinase A enhances SOX9's ability to transactivate a Col2a1 chondrocyte-specific enhancer. Mol Cell Biol. 2000;20:4149-58 pubmed
  15. Mori Akiyama Y, van den Born M, van Es J, Hamilton S, Adams H, Zhang J, et al. SOX9 is required for the differentiation of paneth cells in the intestinal epithelium. Gastroenterology. 2007;133:539-46 pubmed
    ..We conclude that SOX9 is required for the differentiation of Paneth cells. Our results elucidate an essential step in the differentiation of gut epithelium. ..
  16. Akiyama H, Kim J, Nakashima K, Balmes G, Iwai N, Deng J, et al. Osteo-chondroprogenitor cells are derived from Sox9 expressing precursors. Proc Natl Acad Sci U S A. 2005;102:14665-70 pubmed
    ..Thus, our results strongly suggest that all osteo-chondroprogenitor cells, as well as progenitors in a variety of tissues, are derived from Sox9-expressing precursors during mouse embryogenesis. ..
  17. Qin Y, Bishop C. Sox9 is sufficient for functional testis development producing fertile male mice in the absence of Sry. Hum Mol Genet. 2005;14:1221-9 pubmed
  18. Akiyama H, Kamitani T, Yang X, Kandyil R, Bridgewater L, Fellous M, et al. The transcription factor Sox9 is degraded by the ubiquitin-proteasome system and stabilized by a mutation in a ubiquitin-target site. Matrix Biol. 2005;23:499-505 pubmed
    ..Our in vitro studies indicate that the ubiquitin-proteasome proteolytic system degrades Sox9 and regulates its transcriptional activity. ..
  19. Bi W, Deng J, Zhang Z, Behringer R, de Crombrugghe B. Sox9 is required for cartilage formation. Nat Genet. 1999;22:85-9 pubmed
    ..Moreover, no cartilage developed in teratomas derived from Sox9-/- embryonic stem (ES) cells. Our results identify Sox9 as the first transcription factor that is essential for chondrocyte differentiation and cartilage formation. ..
  20. Bridgewater L, Lefebvre V, de Crombrugghe B. Chondrocyte-specific enhancer elements in the Col11a2 gene resemble the Col2a1 tissue-specific enhancer. J Biol Chem. 1998;273:14998-5006 pubmed
    ..These similarities suggest the existence of a genetic program designed to coordinately regulate the expression of these and perhaps other genes involved in the chondrocyte differentiation pathway. ..
  21. Smits P, Li P, Mandel J, Zhang Z, Deng J, Behringer R, et al. The transcription factors L-Sox5 and Sox6 are essential for cartilage formation. Dev Cell. 2001;1:277-90 pubmed
    ..L-Sox5 and Sox6 are thus redundant, potent enhancers of chondroblast functions, thereby essential for endochondral skeleton formation. ..
  22. Lefebvre V, Huang W, Harley V, Goodfellow P, de Crombrugghe B. SOX9 is a potent activator of the chondrocyte-specific enhancer of the pro alpha1(II) collagen gene. Mol Cell Biol. 1997;17:2336-46 pubmed
    ..We speculate that in campomelic dysplasia a decrease in SOX9 activity would inhibit production of collagen II, and eventually other cartilage matrix proteins, leading to major skeletal anomalies. ..
  23. Akiyama H, Lyons J, Mori Akiyama Y, Yang X, Zhang R, Zhang Z, et al. Interactions between Sox9 and beta-catenin control chondrocyte differentiation. Genes Dev. 2004;18:1072-87 pubmed
    ..Our results strongly suggest that chondrogenesis is controlled by interactions between Sox9 and the Wnt/beta-catenin signaling pathway. ..
  24. Lefebvre V, Behringer R, de Crombrugghe B. L-Sox5, Sox6 and Sox9 control essential steps of the chondrocyte differentiation pathway. Osteoarthritis Cartilage. 2001;9 Suppl A:S69-75 pubmed
    ..Their discovery will help to understand further the molecular mechanisms controlling chondrogenesis in vivo, uncover genetic mechanisms underlying cartilage diseases, and develop novel strategies for cartilage repair. ..
  25. Poche R, Furuta Y, Chaboissier M, Schedl A, Behringer R. Sox9 is expressed in mouse multipotent retinal progenitor cells and functions in Müller glial cell development. J Comp Neurol. 2008;510:237-50 pubmed publisher
    ..Furthermore, by employing a conditional knockout approach, we show that Sox9 is essential for the differentiation and/or survival of postnatal Müller glial cells. ..
  26. Chen Q, Dowhan D, Liang D, Moore D, Overbeek P. CREB-binding protein/p300 co-activation of crystallin gene expression. J Biol Chem. 2002;277:24081-9 pubmed
    ..In a promoter-specific fashion, co-activation can be modulated by Prox-1 and/or Sox-1. This modulation may help to specify the endogenous levels of crystallin gene expression. ..
  27. Hattori T, Eberspaecher H, Lu J, Zhang R, Nishida T, Kahyo T, et al. Interactions between PIAS proteins and SOX9 result in an increase in the cellular concentrations of SOX9. J Biol Chem. 2006;281:14417-28 pubmed
    ..Our results suggest that, by controlling the cellular concentrations of SOX9, PIAS proteins and sumoylation may be part of a major regulatory system of SOX9 functions. ..
  28. Arango N, Lovell Badge R, Behringer R. Targeted mutagenesis of the endogenous mouse Mis gene promoter: in vivo definition of genetic pathways of vertebrate sexual development. Cell. 1999;99:409-19 pubmed
    ..Comparative studies of Mis expression in vertebrates indicate that the Mis promoter receives transcriptional inputs that vary between species but result in the same functional readout...
  29. Akiyama H, Chaboissier M, Behringer R, Rowitch D, Schedl A, Epstein J, et al. Essential role of Sox9 in the pathway that controls formation of cardiac valves and septa. Proc Natl Acad Sci U S A. 2004;101:6502-7 pubmed
    ..We propose that loss of Sox9 inhibits epithelial-mesenchymal transformation after delamination and initial migration, but before definitive mesenchymal transformation. ..
  30. ACEVEDO V, Gangula R, Freeman K, Li R, Zhang Y, Wang F, et al. Inducible FGFR-1 activation leads to irreversible prostate adenocarcinoma and an epithelial-to-mesenchymal transition. Cancer Cell. 2007;12:559-71 pubmed
    ..The iFGFR1 model clearly implicates FGFR1 in PCa progression and demonstrates how CID-inducible models can help evaluate candidate molecules in tumor progression and maintenance...