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S L Hofmann Selected Research

palmitoyl-protein thioesterase

2/2006Inefficient cleavage of palmitoyl-protein thioesterase (PPT) substrates by aminothiols: implications for treatment of infantile neuronal ceroid lipofuscinosis.
6/2001Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis.
1/2001Positional candidate gene cloning of CLN1.
4/2000The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis.

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S L Hofmann Research Topics

Disease

7Neuronal Ceroid-Lipofuscinoses (Neuronal Ceroid Lipofuscinosis)
02/2006 - 02/2000
5Neurodegenerative Diseases (Neurodegenerative Disease)
02/2006 - 04/2000
1Cystinosis
02/2006

Drug/Important Bio-Agent (IBA)

4EnzymesIBA
02/2006 - 04/2000
4palmitoyl-protein thioesteraseIBA
02/2006 - 04/2000
3Proteins (Proteins, Gene)FDA Link
02/2006 - 02/2000
2Cysteine (L-Cysteine)FDA Link
02/2006 - 04/2000
1Cysteamine (Mercaptamine)FDA Link
02/2006
1Fatty Acids (Saturated Fatty Acids)IBA
02/2006
1Palmitoyl Coenzyme A (Palmitoyl CoA)IBA
02/2006
1Pharmaceutical PreparationsIBA
02/2006
1Cystine (L-Cystine)IBA
02/2006
1LipofuscinIBA
01/2001