Aurora Pujol

Summary

Country: France

Publications

  1. ncbi Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy
    Aurora Pujol
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM ULP, B P 163, 67404 Illkirch Cedex, C U de Strasbourg, France
    Hum Mol Genet 11:499-505. 2002
  2. ncbi Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy
    Aurora Pujol
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM ULP and Collè de France Illkirch, CU de Strasbourg, France
    Hum Mol Genet 13:2997-3006. 2004
  3. ncbi Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage
    Isidre Ferrer
    Institut de Neuropatologia, Hospital Universitari de Bellvitge, Department de Biologia Cel lular i Anatomia Patologica, Facultat de Medicina, Universitat de Barcelona, Spain
    Hum Mol Genet 14:3565-77. 2005
  4. ncbi The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage
    Jyh Feng Lu
    Johns Hopkins School of Medicine, Kennedy Krieger Institute, Baltimore, MD, USA
    Lab Invest 87:261-72. 2007
  5. doi Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy
    Stephane Fourcade
    Centre de Genètica Mèdica i Molecular, Institut d Investigació Biomèdica de Bellvitge IDIBELL, Hospitalet de Llobregat, Barcelona, Spain
    Hum Mol Genet 17:1762-73. 2008
  6. ncbi Mouse liver PMP70 and ALDP: homomeric interactions prevail in vivo
    Carla P Guimaraes
    Instituto de Biologia Molecular e Celular, Rua do Campo Alegre, Porto, Portugal
    Biochim Biophys Acta 1689:235-43. 2004

Collaborators

  • Marisa Giros
  • Isidre Ferrer
  • S Kemp
  • J M Powers
  • Ronald J A Wanders
  • Ann K Heinzer
  • Stephane Fourcade
  • Jyh Feng Lu
  • Carla P Guimaraes
  • Jone López-Erauskin
  • Reinald Pamplona
  • Alba Naudi
  • Jorge Galino
  • Manuel Portero-Otin
  • Mariona Jove
  • Francesc Villarroya
  • Carine Duval
  • Karen L deMesy Bentley
  • Rebecca Deering
  • Emily Barron-Casella
  • Zhengtong Pei
  • Gary S Wand
  • Martina C McGuinness
  • Paul A Watkins
  • Kirby D Smith
  • Ann B Moser
  • Patrick Aubourg
  • Clara Sa-Miranda
  • Françoise Fouquet
  • Jorge E Azevedo
  • Gerardo Jimenez-Sanchez
  • Pedro Domingues

Detail Information

Publications6

  1. ncbi Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy
    Aurora Pujol
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM ULP, B P 163, 67404 Illkirch Cedex, C U de Strasbourg, France
    Hum Mol Genet 11:499-505. 2002
    ..The phenotype of ALD-deficient mice mimics features of human AMN, thus providing a model for investigating the pathogenesis of this disease...
  2. ncbi Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy
    Aurora Pujol
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM ULP and Collè de France Illkirch, CU de Strasbourg, France
    Hum Mol Genet 13:2997-3006. 2004
    ..Thus, our results provide direct evidence for functional redundancy/overlap between both transporters in vivo and highlight ALDR as therapeutic target for treatment of X-ALD...
  3. ncbi Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage
    Isidre Ferrer
    Institut de Neuropatologia, Hospital Universitari de Bellvitge, Department de Biologia Cel lular i Anatomia Patologica, Facultat de Medicina, Universitat de Barcelona, Spain
    Hum Mol Genet 14:3565-77. 2005
    ..We have identified mitochondrial, Golgi and endoplasmic reticulum damage as the underlying pathological mechanism, thus providing evidence of a disturbed organelle cross-talk, which may be at the origin of the pathological cascade...
  4. ncbi The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage
    Jyh Feng Lu
    Johns Hopkins School of Medicine, Kennedy Krieger Institute, Baltimore, MD, USA
    Lab Invest 87:261-72. 2007
    ....
  5. doi Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy
    Stephane Fourcade
    Centre de Genètica Mèdica i Molecular, Institut d Investigació Biomèdica de Bellvitge IDIBELL, Hospitalet de Llobregat, Barcelona, Spain
    Hum Mol Genet 17:1762-73. 2008
    ..These results pave the way for the identification of therapeutic targets that could reverse the deregulated response to oxidative stress in X-ALD...
  6. ncbi Mouse liver PMP70 and ALDP: homomeric interactions prevail in vivo
    Carla P Guimaraes
    Instituto de Biologia Molecular e Celular, Rua do Campo Alegre, Porto, Portugal
    Biochim Biophys Acta 1689:235-43. 2004
    ..Our data indicate that the majority (if not all) of mouse liver PMP70 and ALDP are homomeric proteins...