Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
1R01NS035323-01
Application #
2274630
Study Section
Special Emphasis Panel (ZRG1-NLS-3 (01))
Project Start
1996-07-01
Project End
2000-05-31
Budget Start
1996-07-01
Budget End
1997-05-31
Support Year
1
Fiscal Year
1996
Total Cost
Indirect Cost

  Institution

Name
University of Texas Sw Medical Center Dallas
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
City
Dallas
State
TX
Country
United States
Zip Code
75390

  Publications

Cho, Steve K; Hofmann, Sandra L (2004) pdf1, a palmitoyl protein thioesterase 1 Ortholog in Schizosaccharomyces pombe: a yeast model of infantile Batten disease. Eukaryot Cell 3:302-10
Calero, Guillermo; Gupta, Praveena; Nonato, M Cristina et al. (2003) The crystal structure of palmitoyl protein thioesterase-2 (PPT2) reveals the basis for divergent substrate specificities of the two lysosomal thioesterases, PPT1 and PPT2. J Biol Chem 278:37957-64
Lu, Jui-Yun; Verkruyse, Linda A; Hofmann, Sandra L (2002) The effects of lysosomotropic agents on normal and INCL cells provide further evidence for the lysosomal nature of palmitoyl-protein thioesterase function. Biochim Biophys Acta 1583:35-44
Hofmann, Sandra L; Atashband, Armita; Cho, Steve K et al. (2002) Neuronal ceroid lipofuscinoses caused by defects in soluble lysosomal enzymes (CLN1 and CLN2). Curr Mol Med 2:423-37
Rush, Jeffrey S; Cho, Steve K; Jiang, Songmin et al. (2002) Identification and characterization of a cDNA encoding a dolichyl pyrophosphate phosphatase located in the endoplasmic reticulum of mammalian cells. J Biol Chem 277:45226-34
Gupta, P; Hofmann, S L (2002) Neuronal ceroid lipofuscinosis/Batten disease: the lysosomal proteinoses. Mol Psychiatry 7:434-6
Hofmann, S L; Das, A K; Lu, J Y et al. (2001) Positional candidate gene cloning of CLN1. Adv Genet 45:69-92
Das, A K; Bellizzi 3rd, J J; Tandel, S et al. (2000) Structural basis for the insensitivity of a serine enzyme (palmitoyl-protein thioesterase) to phenylmethylsulfonyl fluoride. J Biol Chem 275:23847-51
Bellizzi 3rd, J J; Widom, J; Kemp, C et al. (2000) The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis. Proc Natl Acad Sci U S A 97:4573-8
Hofmann, S L; Verkruyse, L A (1999) Fatty acid analysis of protein-derived lipid thioesters isolated from palmitoyl-protein thioesterase-deficient cells. Methods Mol Biol 116:221-8

Showing the most recent 10 out of 16 publications