Abstract

X-linked adrenoleukodystrophy (ALD) is a post-natal neurodegenerative disorder characterized biochemically by elevated levels od very long chain fatty acids due to their defective degradation in peroxisomes. The childhood form of ALD has characteristic cerebral demyelinating lesions associated with perivascular inflammation. The adult form has little in any cerebral inflammation and does not involve the brain. We have generated a potential mouse model for ALD by targeted gene disruption in order to investigate ALD pathophysiology, genetics, and therapy. As an aid to validating the mouse model, to follow disease progression, and to evaluate potential therapeutic intervention, we would like to have a series of MRIs performed on treated and untreated ALD mice with the defective ALD gene in different genetic backgrounds. Dr. Reiss is experienced in the interpretation of MRIs of human cerebral ALD and the analytical programs developed in his neuroimaging center will be available for his project.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
Biotechnology Resource Grants (P41)
Project #
5P41RR005959-12
Application #
6493763
Study Section
Project Start
2001-09-01
Project End
2002-08-31
Budget Start
Budget End
Support Year
12
Fiscal Year
2001
Total Cost
$288,015
Indirect Cost

  Institution

Name
Duke University
Department
Type
DUNS #
071723621
City
Durham
State
NC
Country
United States
Zip Code
27705

  Publications

Tang, Xinyan; Jing, Liufang; Richardson, William J et al. (2016) Identifying molecular phenotype of nucleus pulposus cells in human intervertebral disc with aging and degeneration. J Orthop Res 34:1316-26
Hodgkinson, Conrad P; Bareja, Akshay; Gomez, José A et al. (2016) Emerging Concepts in Paracrine Mechanisms in Regenerative Cardiovascular Medicine and Biology. Circ Res 118:95-107
Schmeckpeper, Jeffrey; Verma, Amanda; Yin, Lucy et al. (2015) Inhibition of Wnt6 by Sfrp2 regulates adult cardiac progenitor cell differentiation by differential modulation of Wnt pathways. J Mol Cell Cardiol 85:215-25
Roos, Justus E; McAdams, Holman P; Kaushik, S Sivaram et al. (2015) Hyperpolarized Gas MR Imaging: Technique and Applications. Magn Reson Imaging Clin N Am 23:217-29
He, Mu; Robertson, Scott H; Kaushik, S Sivaram et al. (2015) Dose and pulse sequence considerations for hyperpolarized (129)Xe ventilation MRI. Magn Reson Imaging 33:877-85
Huang, Lingling; Walter, Vonn; Hayes, D Neil et al. (2014) Hedgehog-GLI signaling inhibition suppresses tumor growth in squamous lung cancer. Clin Cancer Res 20:1566-75
Huang, Jing; Guo, Jian; Beigi, Farideh et al. (2014) HASF is a stem cell paracrine factor that activates PKC epsilon mediated cytoprotection. J Mol Cell Cardiol 66:157-64
Yuan, Ying; Gilmore, John H; Geng, Xiujuan et al. (2014) FMEM: functional mixed effects modeling for the analysis of longitudinal white matter Tract data. Neuroimage 84:753-64
He, Mu; Kaushik, S Sivaram; Robertson, Scott H et al. (2014) Extending semiautomatic ventilation defect analysis for hyperpolarized (129)Xe ventilation MRI. Acad Radiol 21:1530-41
van Rhoon, Gerard C; Samaras, Theodoros; Yarmolenko, Pavel S et al. (2013) CEM43°C thermal dose thresholds: a potential guide for magnetic resonance radiofrequency exposure levels? Eur Radiol 23:2215-27

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