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Gene Review

Ds  -  disorganization

Mus musculus

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Disease relevance of Ds

  • Although other mouse mutations produce similar congenital defects, extreme pleiotropism, random occurrence, developmental independence of multiple defects, and type of anomaly make Ds unique [1].
  • Here, we show that Runx1 is required to sustain muscle by preventing denervated myofibers from undergoing myofibrillar disorganization and autophagy, structural defects found in a variety of congenital myopathies [2].
  • Islet disorganization and fibrosis did not result from lymphocytic infiltration, since they were also observed in SCID mice bearing the transgene [3].
  • We report the generation of Cd151-null mice that recapitulate the renal pathology of human patients, i.e., with age they develop massive proteinuria caused by focal glomerulosclerosis, disorganization of the glomerular basement membrane, and tubular cystic dilation [4].
  • Normoleptinemic control ACS-transgenic mice developed severe dilated cardiomyopathy with thickened left ventricular walls and profound impairment of systolic function on echocardiogram; histologically, there was severe myofiber disorganization and interstitial fibrosis, with intracytoplasmic lipid vacuoles identifiable by electron microscope [5].

Psychiatry related information on Ds


High impact information on Ds


Chemical compound and disease context of Ds


Biological context of Ds

  • This mapping assignment places the Gata4 gene in the vicinity of the mouse Ds (disorganization) locus, a dominant gain-of-function mutation affecting embryonic development [18].
  • Ds may therefore serve not only as a model for developmental anomalies in cell fate and pattern formation but also for complex developmental traits showing variable expressivity, low penetrance, and sporadic occurrence in mice and humans [19].
  • To characterize the effects of gene dosage, we examined the viability and phenotype of Ds homozygotes and the phenotype of +/+/Ds trisomic fetuses [1].
  • Not only were Ds homozygotes found in their expected frequency, homozygotes were not more severely affected than heterozygotes [1].
  • Trisomies provide a direct test for determining whether Ds is a gain-of-function mutation [1].

Anatomical context of Ds

  • Two trisomic fetuses had developmental defects characteristic of Ds mice [1].
  • Rho+/- animals retain the majority of their photoreceptors although the inner and outer segments of these cells display some structural disorganization, the outer segments becoming shorter in older mice [20].
  • Most mice lacking Mef2a died suddenly within the first week of life and exhibited pronounced dilation of the right ventricle, myofibrillar fragmentation, mitochondrial disorganization and activation of a fetal cardiac gene program [21].
  • Null mice display ataxia, behavioral abnormalities, and tremors that correlate with a twofold decrease in brain mass that disproportionately affects the cerebellum (sixfold reduced in mass) and the cerebral cortex, both of which show signs of disorganization [22].
  • Runx1 prevents wasting, myofibrillar disorganization, and autophagy of skeletal muscle [2].

Associations of Ds with chemical compounds


Physical interactions of Ds


Regulatory relationships of Ds


Other interactions of Ds

  • Unlike the two-hit model that has been used to describe the development of retinoblastoma, the "second hit" for Ds is thought not to arise in the other Ds allele [32].
  • Although the tensile properties of the wounds were unchanged, ultrastructural analysis showed a significantly decreased level of debridement, greater disorganization of matrix, and an alteration of collagen fibrillogenesis leading to small diameter collagen fibrils in the OPN mutant mice [33].
  • Cystic embryoid bodies derived from afadin(-/-) embryonic stem cells showed normal organization of the endoderm but disorganization of the ectoderm [34].
  • However, after this time, marked disorganization of the vascular system was observed in those chimeric mice that have a high contribution of Lmo2-null ES cells [35].
  • In this model, Capn3 proteolytic activity leads to disruption of the actin cytoskeleton and disorganization of focal adhesions through cleavage of several endogenous proteins [36].

Analytical, diagnostic and therapeutic context of Ds


  1. Disorganization is a completely dominant gain-of-function mouse mutation causing sporadic developmental defects. Crosby, J.L., Varnum, D.S., Washburn, L.L., Nadeau, J.H. Mech. Dev. (1992) [Pubmed]
  2. Runx1 prevents wasting, myofibrillar disorganization, and autophagy of skeletal muscle. Wang, X., Blagden, C., Fan, J., Nowak, S.J., Taniuchi, I., Littman, D.R., Burden, S.J. Genes Dev. (2005) [Pubmed]
  3. Expression of a tumor necrosis factor alpha transgene in murine pancreatic beta cells results in severe and permanent insulitis without evolution towards diabetes. Higuchi, Y., Herrera, P., Muniesa, P., Huarte, J., Belin, D., Ohashi, P., Aichele, P., Orci, L., Vassalli, J.D., Vassalli, P. J. Exp. Med. (1992) [Pubmed]
  4. Kidney failure in mice lacking the tetraspanin CD151. Sachs, N., Kreft, M., van den Bergh Weerman, M.A., Beynon, A.J., Peters, T.A., Weening, J.J., Sonnenberg, A. J. Cell Biol. (2006) [Pubmed]
  5. Hyperleptinemia prevents lipotoxic cardiomyopathy in acyl CoA synthase transgenic mice. Lee, Y., Naseem, R.H., Duplomb, L., Park, B.H., Garry, D.J., Richardson, J.A., Schaffer, J.E., Unger, R.H. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  6. Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients. Sathasivam, K., Woodman, B., Mahal, A., Bertaux, F., Wanker, E.E., Shima, D.T., Bates, G.P. Hum. Mol. Genet. (2001) [Pubmed]
  7. Pathogenicity of Spiroplasma sp. strain SMCA in Syrian hamsters: clinical, microbiological, and histological aspects. Kirchhoff, H., Kuwabara, T., Barile, M.F. Infect. Immun. (1981) [Pubmed]
  8. Serine 732 phosphorylation of FAK by Cdk5 is important for microtubule organization, nuclear movement, and neuronal migration. Xie, Z., Sanada, K., Samuels, B.A., Shih, H., Tsai, L.H. Cell (2003) [Pubmed]
  9. Mutation of CDH23, encoding a new member of the cadherin gene family, causes Usher syndrome type 1D. Bolz, H., von Brederlow, B., Ramírez, A., Bryda, E.C., Kutsche, K., Nothwang, H.G., Seeliger, M., del C-Salcedó Cabrera, M., Vila, M.C., Molina, O.P., Gal, A., Kubisch, C. Nat. Genet. (2001) [Pubmed]
  10. Perlecan is essential for cartilage and cephalic development. Arikawa-Hirasawa, E., Watanabe, H., Takami, H., Hassell, J.R., Yamada, Y. Nat. Genet. (1999) [Pubmed]
  11. Graded reduction of Pafah1b1 (Lis1) activity results in neuronal migration defects and early embryonic lethality. Hirotsune, S., Fleck, M.W., Gambello, M.J., Bix, G.J., Chen, A., Clark, G.D., Ledbetter, D.H., McBain, C.J., Wynshaw-Boris, A. Nat. Genet. (1998) [Pubmed]
  12. Cardiovascular anomaly, impaired actin bundling and resistance to Src-induced transformation in mice lacking p130Cas. Honda, H., Oda, H., Nakamoto, T., Honda, Z., Sakai, R., Suzuki, T., Saito, T., Nakamura, K., Nakao, K., Ishikawa, T., Katsuki, M., Yazaki, Y., Hirai, H. Nat. Genet. (1998) [Pubmed]
  13. Targeting and processing of glycophorins in murine erythroleukemia cells: use of brefeldin A as a perturbant of intracellular traffic. Ulmer, J.B., Palade, G.E. Proc. Natl. Acad. Sci. U.S.A. (1989) [Pubmed]
  14. Angiogenic acceleration of Neu induced mammary tumor progression and metastasis. Oshima, R.G., Lesperance, J., Munoz, V., Hebbard, L., Ranscht, B., Sharan, N., Muller, W.J., Hauser, C.A., Cardiff, R.D. Cancer Res. (2004) [Pubmed]
  15. Cytokine kinetics and other host factors in response to pneumococcal pulmonary infection in mice. Bergeron, Y., Ouellet, N., Deslauriers, A.M., Simard, M., Olivier, M., Bergeron, M.G. Infect. Immun. (1998) [Pubmed]
  16. Thiamine deficiency in cultured neuroblastoma cells: effect on mitochondrial function and peripheral benzodiazepine receptors. Bettendorff, L., Goessens, G., Sluse, F., Wins, P., Bureau, M., Laschet, J., Grisar, T. J. Neurochem. (1995) [Pubmed]
  17. alpha-Lipoic acid prevents lipotoxic cardiomyopathy in acyl CoA-synthase transgenic mice. Lee, Y., Naseem, R.H., Park, B.H., Garry, D.J., Richardson, J.A., Schaffer, J.E., Unger, R.H. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  18. Assignment of the transcription factor GATA4 gene to human chromosome 8 and mouse chromosome 14: Gata4 is a candidate gene for Ds (disorganization). White, R.A., Dowler, L.L., Pasztor, L.M., Gatson, L.L., Adkison, L.R., Angeloni, S.V., Wilson, D.B. Genomics (1995) [Pubmed]
  19. Two-hit model for sporadic congenital anomalies in mice with the disorganization mutation. Crosby, J.L., Varnum, D.S., Nadeau, J.H. Am. J. Hum. Genet. (1993) [Pubmed]
  20. Retinopathy induced in mice by targeted disruption of the rhodopsin gene. Humphries, M.M., Rancourt, D., Farrar, G.J., Kenna, P., Hazel, M., Bush, R.A., Sieving, P.A., Sheils, D.M., McNally, N., Creighton, P., Erven, A., Boros, A., Gulya, K., Capecchi, M.R., Humphries, P. Nat. Genet. (1997) [Pubmed]
  21. Mitochondrial deficiency and cardiac sudden death in mice lacking the MEF2A transcription factor. Naya, F.J., Black, B.L., Wu, H., Bassel-Duby, R., Richardson, J.A., Hill, J.A., Olson, E.N. Nat. Med. (2002) [Pubmed]
  22. N-myc is essential during neurogenesis for the rapid expansion of progenitor cell populations and the inhibition of neuronal differentiation. Knoepfler, P.S., Cheng, P.F., Eisenman, R.N. Genes Dev. (2002) [Pubmed]
  23. Mannose-induced dysmorphogenesis of metanephric kidney. Role of proteoglycans and adenosine triphosphate. Liu, Z.Z., Carone, F.A., Dalecki, T.M., Lelongt, B., Wallner, E.I., Kanwar, Y.S. J. Clin. Invest. (1992) [Pubmed]
  24. Electric field-directed cell shape changes, displacement, and cytoskeletal reorganization are calcium dependent. Onuma, E.K., Hui, S.W. J. Cell Biol. (1988) [Pubmed]
  25. 3-Deazaadenosine-induced disorganization of macrophage microfilaments. Stopford, C.R., Wolberg, G., Prus, K.L., Reynolds-Vaughn, R., Zimmerman, T.P. Proc. Natl. Acad. Sci. U.S.A. (1985) [Pubmed]
  26. Nucleocytoplasmic shuttling of STK16 (PKL12), a Golgi-resident serine/threonine kinase involved in VEGF expression regulation. Guinea, B., Ligos, J.M., Laín de Lera, T., Martín-Caballero, J., Flores, J., Gonzalez de la Peña, M., García-Castro, J., Bernad, A. Exp. Cell Res. (2006) [Pubmed]
  27. T-tubule disorganization and reduced synchrony of Ca2+ release in murine cardiomyocytes following myocardial infarction. Louch, W.E., Mørk, H.K., Sexton, J., Strømme, T.A., Laake, P., Sjaastad, I., Sejersted, O.M. J. Physiol. (Lond.) (2006) [Pubmed]
  28. The dystrophin complex forms a mechanically strong link between the sarcolemma and costameric actin. Rybakova, I.N., Patel, J.R., Ervasti, J.M. J. Cell Biol. (2000) [Pubmed]
  29. Omomyc, a potential Myc dominant negative, enhances Myc-induced apoptosis. Soucek, L., Jucker, R., Panacchia, L., Ricordy, R., Tatò, F., Nasi, S. Cancer Res. (2002) [Pubmed]
  30. A requirement for neuropilin-1 in embryonic vessel formation. Kawasaki, T., Kitsukawa, T., Bekku, Y., Matsuda, Y., Sanbo, M., Yagi, T., Fujisawa, H. Development (1999) [Pubmed]
  31. Stimulus-dependent disorganization of actin filaments induced by overexpression of cofilin in C2 myoblasts. Ono, S., Abe, H., Obinata, T. Cell Struct. Funct. (1996) [Pubmed]
  32. Disorganization in mice and humans. Robin, N.H., Nadeau, J.H. Am. J. Med. Genet. (2001) [Pubmed]
  33. Altered wound healing in mice lacking a functional osteopontin gene (spp1). Liaw, L., Birk, D.E., Ballas, C.B., Whitsitt, J.S., Davidson, J.M., Hogan, B.L. J. Clin. Invest. (1998) [Pubmed]
  34. Afadin: A key molecule essential for structural organization of cell-cell junctions of polarized epithelia during embryogenesis. Ikeda, W., Nakanishi, H., Miyoshi, J., Mandai, K., Ishizaki, H., Tanaka, M., Togawa, A., Takahashi, K., Nishioka, H., Yoshida, H., Mizoguchi, A., Nishikawa, S., Takai, Y. J. Cell Biol. (1999) [Pubmed]
  35. The oncogenic LIM-only transcription factor Lmo2 regulates angiogenesis but not vasculogenesis in mice. Yamada, Y., Pannell, R., Forster, A., Rabbitts, T.H. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  36. Calpain 3 is activated through autolysis within the active site and lyses sarcomeric and sarcolemmal components. Taveau, M., Bourg, N., Sillon, G., Roudaut, C., Bartoli, M., Richard, I. Mol. Cell. Biol. (2003) [Pubmed]
  37. Inhibition of DNA synthesis and neoplastic cell growth by vitamin A (retinol). Lupulescu, A. J. Natl. Cancer Inst. (1986) [Pubmed]
  38. Mouse Polycomb M33 is required for splenic vascular and adrenal gland formation through regulating Ad4BP/SF1 expression. Katoh-Fukui, Y., Owaki, A., Toyama, Y., Kusaka, M., Shinohara, Y., Maekawa, M., Toshimori, K., Morohashi, K. Blood (2005) [Pubmed]
  39. Hemorrhage, impaired hematopoiesis, and lethality in mouse embryos carrying a targeted disruption of the Fli1 transcription factor. Spyropoulos, D.D., Pharr, P.N., Lavenburg, K.R., Jackers, P., Papas, T.S., Ogawa, M., Watson, D.K. Mol. Cell. Biol. (2000) [Pubmed]
  40. Astrotactin provides a receptor system for CNS neuronal migration. Fishell, G., Hatten, M.E. Development (1991) [Pubmed]
  41. Extracellular proteolysis alters tooth development in transgenic mice expressing urokinase-type plasminogen activator in the enamel organ. Zhou, H.M., Nichols, A., Wohlwend, A., Bolon, I., Vassalli, J.D. Development (1999) [Pubmed]
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