Hofmann SL - Search Results

1

Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis.

Das AK, Lu JY, Hofmann SL. Das AK, et al. Among authors: hofmann sl. Hum Mol Genet. 2001 Jun 15;10(13):1431-9. doi: 10.1093/hmg/10.13.1431. Hum Mol Genet. 2001. PMID: 11440996

2

The effects of lysosomotropic agents on normal and INCL cells provide further evidence for the lysosomal nature of palmitoyl-protein thioesterase function.

Lu JY, Verkruyse LA, Hofmann SL. Lu JY, et al. Among authors: hofmann sl. Biochim Biophys Acta. 2002 Jun 13;1583(1):35-44. doi: 10.1016/s1388-1981(02)00158-0. Biochim Biophys Acta. 2002. PMID: 12069847

3

Neuronal ceroid lipofuscinoses caused by defects in soluble lysosomal enzymes (CLN1 and CLN2).

Hofmann SL, Atashband A, Cho SK, Das AK, Gupta P, Lu JY. Hofmann SL, et al. Curr Mol Med. 2002 Aug;2(5):423-37. doi: 10.2174/1566524023362294. Curr Mol Med. 2002. PMID: 12125808 Review.

4

Thematic review series: lipid posttranslational modifications. Lysosomal metabolism of lipid-modified proteins.

Lu JY, Hofmann SL. Lu JY, et al. Among authors: hofmann sl. J Lipid Res. 2006 Jul;47(7):1352-7. doi: 10.1194/jlr.R600010-JLR200. Epub 2006 Apr 20. J Lipid Res. 2006. PMID: 16627894 Free article. Review.

5

Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory response.

Qiao X, Lu JY, Hofmann SL. Qiao X, et al. Among authors: hofmann sl. BMC Neurosci. 2007 Nov 16;8:95. doi: 10.1186/1471-2202-8-95. BMC Neurosci. 2007. PMID: 18021406 Free PMC article.

6

Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis.

Lu JY, Hu J, Hofmann SL. Lu JY, et al. Among authors: hofmann sl. Mol Genet Metab. 2010 Apr;99(4):374-8. doi: 10.1016/j.ymgme.2009.12.002. Epub 2009 Dec 5. Mol Genet Metab. 2010. PMID: 20036592 Free PMC article.

7

Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.

Hu J, Lu JY, Wong AM, Hynan LS, Birnbaum SG, Yilmaz DS, Streit BM, Lenartowicz EM, Thompson TC, Cooper JD, Hofmann SL. Hu J, et al. Among authors: hofmann sl. Mol Genet Metab. 2012 Sep;107(1-2):213-21. doi: 10.1016/j.ymgme.2012.05.009. Epub 2012 May 22. Mol Genet Metab. 2012. PMID: 22704978 Free PMC article.

8

Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.

Lu JY, Nelvagal HR, Wang L, Birnbaum SG, Cooper JD, Hofmann SL. Lu JY, et al. Among authors: hofmann sl. Mol Genet Metab. 2015 Sep-Oct;116(1-2):98-105. doi: 10.1016/j.ymgme.2015.05.005. Epub 2015 May 12. Mol Genet Metab. 2015. PMID: 25982063

9

Histochemical localization of palmitoyl protein thioesterase-1 activity.

Dearborn JT, Ramachandran S, Shyng C, Lu JY, Thornton J, Hofmann SL, Sands MS. Dearborn JT, et al. Among authors: hofmann sl. Mol Genet Metab. 2016 Feb;117(2):210-6. doi: 10.1016/j.ymgme.2015.11.004. Epub 2015 Nov 11. Mol Genet Metab. 2016. PMID: 26597320 Free PMC article.

10

Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase.

Lu JY, Verkruyse LA, Hofmann SL. Lu JY, et al. Among authors: hofmann sl. Proc Natl Acad Sci U S A. 1996 Sep 17;93(19):10046-50. doi: 10.1073/pnas.93.19.10046. Proc Natl Acad Sci U S A. 1996. PMID: 8816748 Free PMC article.

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