Project Start
2000-03-15
Project End
2000-12-31
Budget Start
Budget End
Support Year
23
Fiscal Year
2000
Total Cost
Indirect Cost
Name
Hugo W. Moser Research Institute Kennedy Krieger
Department
Type
DUNS #
167202410
City
Baltimore
State
MD
Country
United States
Zip Code
21205
Brose, Rebecca Deering; Shin, Gloria; McGuinness, Martina C et al. (2012) Activation of the stress proteome as a mechanism for small molecule therapeutics. Hum Mol Genet 21:4237-52
Brose, Rebecca Deering; Avramopoulos, Dimitri; Smith, Kirby D (2012) SOD2 as a potential modifier of X-linked adrenoleukodystrophy clinical phenotypes. J Neurol 259:1440-7
Steinberg, S J; Snowden, A; Braverman, N E et al. (2009) A PEX10 defect in a patient with no detectable defect in peroxisome assembly or metabolism in cultured fibroblasts. J Inherit Metab Dis 32:109-19
Moser, Hugo W; Mahmood, Asif; Raymond, Gerald V (2007) X-linked adrenoleukodystrophy. Nat Clin Pract Neurol 3:140-51
Jia, Zhenzhen; Pei, Zhengtong; Maiguel, Dony et al. (2007) The fatty acid transport protein (FATP) family: very long chain acyl-CoA synthetases or solute carriers? J Mol Neurosci 33:25-31
Watkins, Paul A; Maiguel, Dony; Jia, Zhenzhen et al. (2007) Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome. J Lipid Res 48:2736-50
Jia, Zhenzhen; Moulson, Casey L; Pei, Zhengtong et al. (2007) Fatty acid transport protein 4 is the principal very long chain fatty acyl-CoA synthetase in skin fibroblasts. J Biol Chem 282:20573-83
Eichler, Florian; Mahmood, Asif; Loes, Daniel et al. (2007) Magnetic resonance imaging detection of lesion progression in adult patients with X-linked adrenoleukodystrophy. Arch Neurol 64:659-64
Lu, Jyh-Feng; Barron-Casella, Emily; Deering, Rebecca et al. (2007) The role of peroxisomal ABC transporters in the mouse adrenal gland: the loss of Abcd2 (ALDR), Not Abcd1 (ALD), causes oxidative damage. Lab Invest 87:261-72
Moser, Hugo W (2006) Therapy of X-linked adrenoleukodystrophy. NeuroRx 3:246-53

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