Abstract

A major objective of the cell biology core is to serve as a centralized resource for the establishment or transformation, culture, storage and re-establishment of human cells for the investigations outlined in the various sections of this grant proposal. A second objective is to provide the specialized expertise and equipment required for cytogenetic analysis of cell lines being utilized for these investigations. Finally, FISH analysis will be provided for the mapping of DNA that codes for peroxisomal proteins of interest to the various investigators as outlined elsewhere in this proposal.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Research Program Projects (P01)
Project #
5P01HD010981-22
Application #
6296766
Study Section
Project Start
1999-01-01
Project End
1999-12-31
Budget Start
1998-10-01
Budget End
1999-09-30
Support Year
22
Fiscal Year
1999
Total Cost
Indirect Cost

  Institution

Name
Hugo W. Moser Research Institute Kennedy Krieger
Department
Type
DUNS #
167202410
City
Baltimore
State
MD
Country
United States
Zip Code
21205

  Publications

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
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; 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
Moser, Hugo W (2006) Therapy of X-linked adrenoleukodystrophy. NeuroRx 3:246-53

Showing the most recent 10 out of 167 publications