Y A Ioannou

Summary

Affiliation: Mount Sinai School of Medicine
Country: USA

Publications

  1. ncbi request reprint The structure and function of the Niemann-Pick C1 protein
    Y A Ioannou
    Department of Human Genetics, The Mount Sinai School of Medicine, One Gustave L Levy Place, New York, New York 10029, USA
    Mol Genet Metab 71:175-81. 2000
  2. ncbi request reprint The NPC1 protein: structure implies function
    Catherine Scott
    Department of Human Genetics Box 1498, Mount Sinai School of Medicine, Fifth Avenue at 100th Street, New York, NY 10029, USA
    Biochim Biophys Acta 1685:8-13. 2004
  3. ncbi request reprint Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating
    J P Davies
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York 10029, USA
    J Biol Chem 275:24367-74. 2000
  4. ncbi request reprint Evidence for a Niemann-pick C (NPC) gene family: identification and characterization of NPC1L1
    J P Davies
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York, 10029, USA
    Genomics 65:137-45. 2000
  5. ncbi request reprint Apoptosis-induced release of mature sterol regulatory element-binding proteins activates sterol-responsive genes
    M E Higgins
    Department of Human Genetics, P.O. Box 1498, Mount Sinai School of Medicine, Fifth Avenue at 100th Street, New York, NY 10029, USA
    J Lipid Res 42:1939-46. 2001
  6. ncbi request reprint Multidrug permeases and subcellular cholesterol transport
    Y A Ioannou
    Department of Human Genetics, Box 1498, The Mount Sinai School of Medicine, 1 Gustave L Levy Place, New York, New York 10029, USA
    Nat Rev Mol Cell Biol 2:657-68. 2001
  7. ncbi request reprint Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-Golgi network
    M E Higgins
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Mol Genet Metab 68:1-13. 1999
  8. pmc Fabry disease: preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice
    Y A Ioannou
    Department of Human Genetics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Am J Hum Genet 68:14-25. 2001
  9. pmc Overexpression of human alpha-galactosidase A results in its intracellular aggregation, crystallization in lysosomes, and selective secretion
    Y A Ioannou
    Division of Medical and Molecular Genetics, Mount Sinai School of Medicine, New York 10029
    J Cell Biol 119:1137-50. 1992
  10. pmc Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells
    Y Sugimoto
    Department of Neurobiology, Tottori University Faculty of Medicine, Yonago 683, Japan
    Proc Natl Acad Sci U S A 98:12391-6. 2001

Collaborators

  • R J Desnick
  • B Levy
  • Edward B Neufeld
  • M E Grace
  • J A Morris
  • M Elleder
  • Jerome F Strauss
  • STEPHEN STURLEY
  • Marie T Vanier
  • K Ohno
  • J P Davies
  • M E Higgins
  • Catherine Scott
  • F W Chen
  • A M Wang
  • Y Sugimoto
  • M Ohta
  • E D Carstea
  • K M Zeidner
  • R W Gotlib
  • Y Ohsaki
  • K Higaki
  • H Ninomiya
  • T Ohnishi
  • F Matsuura
  • M E Hodgson
  • M A Rosenfeld
  • D Zhang
  • A Brown
  • R O Brady
  • J Gu
  • D Markie
  • M C Patterson
  • L Liscum
  • J Nagle
  • A Cooney
  • S K Loftus
  • R R O'Neill
  • M H Polymeropoulos
  • D A Tagle
  • D B Krizman
  • C R Kaneski
  • M Zeigler
  • C Cummings
  • J Sokol
  • W J Pavan
  • R Carmi
  • T Y Chang
  • M Comly
  • N K Dwyer
  • E J Blanchette-Mackie
  • K G Coleman
  • O P van Diggelen
  • P G Pentchev
  • C M Disteche
  • D F Bishop
  • D A Adler

Detail Information

Publications16

  1. ncbi request reprint The structure and function of the Niemann-Pick C1 protein
    Y A Ioannou
    Department of Human Genetics, The Mount Sinai School of Medicine, One Gustave L Levy Place, New York, New York 10029, USA
    Mol Genet Metab 71:175-81. 2000
    ..Currently, there is no direct evidence as to the function of the NPC1 protein; however, a number of observations suggest that NPC1 may be related to a family of prokaryotic efflux pumps and thus it may also act as a molecular pump...
  2. ncbi request reprint The NPC1 protein: structure implies function
    Catherine Scott
    Department of Human Genetics Box 1498, Mount Sinai School of Medicine, Fifth Avenue at 100th Street, New York, NY 10029, USA
    Biochim Biophys Acta 1685:8-13. 2004
    ....
  3. ncbi request reprint Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating
    J P Davies
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York 10029, USA
    J Biol Chem 275:24367-74. 2000
    ..Furthermore, our data show that the putative SSD of NPC1 is oriented in the same manner as those of HMG-R and SCAP, providing strong evidence that this domain is functionally important...
  4. ncbi request reprint Evidence for a Niemann-pick C (NPC) gene family: identification and characterization of NPC1L1
    J P Davies
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York, 10029, USA
    Genomics 65:137-45. 2000
    ..Collectively, these data suggest that NPC1L1 and NPC1 form part of a family of related proteins that may have similar functions at different subcellular locations, perhaps at sequential steps of the same cholesterol transport pathway...
  5. ncbi request reprint Apoptosis-induced release of mature sterol regulatory element-binding proteins activates sterol-responsive genes
    M E Higgins
    Department of Human Genetics, P.O. Box 1498, Mount Sinai School of Medicine, Fifth Avenue at 100th Street, New York, NY 10029, USA
    J Lipid Res 42:1939-46. 2001
    ..These results suggest that activation of sterol-responsive genes early during apoptosis may play a role in the proper execution of this program...
  6. ncbi request reprint Multidrug permeases and subcellular cholesterol transport
    Y A Ioannou
    Department of Human Genetics, Box 1498, The Mount Sinai School of Medicine, 1 Gustave L Levy Place, New York, New York 10029, USA
    Nat Rev Mol Cell Biol 2:657-68. 2001
    ..Characterization of their protein products shows that NPC1 and ABCA1 are permeases that belong to two different superfamilies of efflux pumps, which might be important in subcellular lipid and cholesterol transport...
  7. ncbi request reprint Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-Golgi network
    M E Higgins
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Mol Genet Metab 68:1-13. 1999
    ..These data suggest that U18666A may inhibit the retrograde transport of NPC1 from lysosomes to late endosomes for subsequent transfer to the trans-Golgi network...
  8. pmc Fabry disease: preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice
    Y A Ioannou
    Department of Human Genetics, Mount Sinai School of Medicine, New York, NY 10029, USA
    Am J Hum Genet 68:14-25. 2001
    ....
  9. pmc Overexpression of human alpha-galactosidase A results in its intracellular aggregation, crystallization in lysosomes, and selective secretion
    Y A Ioannou
    Division of Medical and Molecular Genetics, Mount Sinai School of Medicine, New York 10029
    J Cell Biol 119:1137-50. 1992
    ..A significant proportion of these enzyme aggregates are unable to bind the M6PR and are selectively secreted via the constitutive secretory pathway, while endogenous lysosomal enzymes bind the M6PRs and are transported to lysosomes...
  10. pmc Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells
    Y Sugimoto
    Department of Neurobiology, Tottori University Faculty of Medicine, Yonago 683, Japan
    Proc Natl Acad Sci U S A 98:12391-6. 2001
    ..These results suggest that transport of CT/GM1 complexes from the early endosome to the plasma membrane depends on the function of NPC1, whereas transport to the Golgi apparatus/endoplasmic reticulum does not...
  11. ncbi request reprint The entire genomic sequence and cDNA expression of mouse alpha-galactosidase A
    R W Gotlib
    Department of Human Genetics, Mount Sinai School of Medicine, New York 10029, USA
    Biochem Mol Med 57:139-48. 1996
    ....
  12. ncbi request reprint Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis
    E D Carstea
    National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Science 277:228-31. 1997
    ....
  13. pmc Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility
    Y A Ioannou
    Department of Human Genetics, Mount Sinai School of Medicine, Fifth Avenue at 100th Street, New York, NY 10029, USA
    Biochem J 332:789-97. 1998
    ....
  14. ncbi request reprint Murine alpha-N-acetylgalactosaminidase: isolation and expression of a full-length cDNA and genomic organization: further evidence of an alpha-galactosidase gene family
    A M Wang
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York, 10029, USA
    Mol Genet Metab 65:165-73. 1998
    ..The availability of the murine gene will permit additional evolutionary comparisons, structure/function analyses, and the generation of mice with alpha-GalNAc deficiency by gene targeting...
  15. ncbi request reprint Human alpha-N-acetylgalactosaminidase: site occupancy and structure of N-linked oligosaccharides
    M Ohta
    Department of Biotechnology, Fukuyama University, Fukuyama, Hiroshima 729 0292, Japan
    Glycobiology 10:251-61. 2000
    ....
  16. ncbi request reprint Differential gene expression in apoptosis: identification of ribosomal protein 23K, a cell proliferation inhibitor
    F W Chen
    Department of Human Genetics, Mount Sinai School of Medicine, New York, New York 10029, USA
    Mol Genet Metab 64:271-82. 1998
    ..Upregulation of 23K expression using a cDNA construct resulted in a decrease in cell proliferation and growth arrest, suggesting a role for 23K protein as a proliferation checkpoint following a cellular insult...