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Featured ArticleNeurobiology of Disease

A Mouse Model of Classical Late-Infantile Neuronal Ceroid Lipofuscinosis Based on Targeted Disruption of the CLN2 Gene Results in a Loss of Tripeptidyl-Peptidase I Activity and Progressive Neurodegeneration

David E. Sleat, Jennifer A. Wiseman, Mukarram El-Banna, Kwi-Hye Kim, Qinwen Mao, Sandy Price, Shannon L. Macauley, Richard L. Sidman, Michael M. Shen, Qi Zhao, Marco A. Passini, Beverly L. Davidson, Gregory R. Stewart and Peter Lobel
Journal of Neuroscience 13 October 2004, 24 (41) 9117-9126; DOI: https://doi.org/10.1523/JNEUROSCI.2729-04.2004
David E. Sleat
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Jennifer A. Wiseman
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Mukarram El-Banna
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Kwi-Hye Kim
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Qinwen Mao
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Sandy Price
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Shannon L. Macauley
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Richard L. Sidman
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Michael M. Shen
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Qi Zhao
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Marco A. Passini
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Beverly L. Davidson
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Gregory R. Stewart
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Peter Lobel
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Article Information

DOI 
https://doi.org/10.1523/JNEUROSCI.2729-04.2004
PubMed 
15483130
Published By 
Society for Neuroscience
History 
  • Received May 19, 2004
  • Revision received August 3, 2004
  • Accepted August 3, 2004
  • First published October 13, 2004.
  • Version of record published October 13, 2004.
Copyright & Usage 
Copyright © 2004 Society for Neuroscience 0270-6474/04/249117-10.00/0

Author Information

  1. David E. Sleat1,2,
  2. Jennifer A. Wiseman1,
  3. Mukarram El-Banna1,
  4. Kwi-Hye Kim1,
  5. Qinwen Mao4,
  6. Sandy Price1,
  7. Shannon L. Macauley5,
  8. Richard L. Sidman6,
  9. Michael M. Shen1,3,
  10. Qi Zhao5,
  11. Marco A. Passini5,
  12. Beverly L. Davidson4,
  13. Gregory R. Stewart5, and
  14. Peter Lobel1,2
  1. 1Center for Advanced Biotechnology and Medicine, and Departments of 2Pharmacology and 3Pediatrics, University of Medicine and Dentistry of New Jersey, Piscataway, New Jersey 08854, 4Department of Internal Medicine, University of Iowa College of Medicine, Iowa City, Iowa 52242, 5Genzyme Corporation, Framingham, Massachusetts 01701, and 6Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02115
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Author contributions

Disclosures

    • Received May 19, 2004.
    • Revision received August 3, 2004.
    • Accepted August 3, 2004.
  • This work was supported by National Institutes of Health Grants NS37918 (P.L.) and HD42837 (M.M.S.). We thank Rosalie Gin, Vivienne Halili, Holly Collins, Jie Bu, and Tatyana Taksir for technical assistance and Istvan Sohar, Li Lin, Thomas Ludwig, John Pintar, Alwin Schuller, and Jixiang Ding for helpful discussions, reagents, and other assistance.

  • Correspondence should be addressed to Peter Lobel or David Sleat, Center for Advanced Biotechnology and Medicine, 679 Hoes Lane, Piscataway, NJ 08854. E-mail: lobel{at}cabm.rutgers.edu or sleat{at}cabm.rutgers.edu.

  • G. R. Stewart's present address: Medtronic Neurological, Columbia Heights, MN 55421.

  • Copyright © 2004 Society for Neuroscience 0270-6474/04/249117-10$15.00/0

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Article usage: October 2004 to March 2021

AbstractFullPdf
Oct 2004200119163
Nov 20044013671
Dec 2004249225
Total 2004264347259
Jan 2005176042
Feb 2005248752
Mar 2005297242
Apr 200534719
May 200573715
Jun 200544522
Jul 2005115223
Aug 2005134418
Sep 2005125114
Oct 2005185623
Nov 2005126029
Dec 2005154118
Total 2005165652317
Jan 200656328
Feb 200683322
Mar 200655926
Apr 200663214
May 200683132
Jun 200654322
Jul 200641714
Aug 20069337
Sep 200642916
Oct 200656415
Nov 2006114010
Dec 200652510
Total 200675469216
Jan 20079289
Feb 200722614
Mar 200783213
Apr 200792418
May 2007103717
Jun 2007101811
Jul 2007123716
Aug 20075186
Sep 2007112525
Oct 200792813
Nov 200782310
Dec 2007112013
Total 2007104316165
Jan 200863419
Feb 200862226
Mar 2008101410
Apr 200861718
May 2008162113
Jun 2008182518
Jul 200871714
Aug 20089246
Sep 2008152414
Oct 2008142110
Nov 2008101310
Dec 2008694
Total 2008123241162
Jan 200962018
Feb 2009103721
Mar 2009112613
Apr 200912158
May 2009102118
Jun 2009111917
Jul 200917225
Aug 20094167
Sep 200941710
Oct 20093175
Nov 200981419
Dec 2009261014
Total 2009122234155
Jan 201081513
Feb 2010122014
Mar 20106108
Apr 2010482
May 2010124111
Jun 201071012
Jul 201092211
Aug 20106113
Sep 2010132311
Oct 2010133713
Nov 201081612
Dec 2010143712
Total 2010112250122
Jan 2011132816
Feb 201121311
Mar 201152310
Apr 2011132516
May 2011122422
Jun 20118269
Jul 20118815
Aug 2011171311
Sep 2011121118
Oct 2011201211
Nov 2011241511
Dec 201191519
Total 2011143213169
Jan 2012172515
Feb 2012172315
Mar 2012302618
Apr 2012181212
May 2012172116
Jun 2012181718
Jul 2012162312
Aug 201292013
Sep 201281414
Oct 201218229
Nov 201236132
Dec 2012331411
Total 2012237230155
Jan 201318178
Feb 2013171311
Mar 201325167
Apr 2013271511
May 2013261210
Jun 20131156
Jul 2013161410
Aug 201312107
Sep 20131688
Oct 2013201117
Nov 201316109
Dec 2013798
Total 2013211140112
Jan 2014222110
Feb 201481014
Mar 2014211011
Apr 201415812
May 2014322337
Jun 201410626
Jul 2014211810
Aug 2014131021
Sep 2014151223
Oct 201471138
Nov 2014162331
Dec 201411339
Total 2014191185242
Jan 2015131212
Feb 201513411
Mar 2015231611
Apr 2015789
May 201562312
Jun 201551714
Jul 201512814
Aug 201561310
Sep 201514259
Oct 201591710
Nov 201512127
Dec 2015191513
Total 2015139170132
Jan 2016302519
Feb 2016162311
Mar 2016262317
Apr 20167168
May 2016132510
Jun 2016112420
Jul 2016201512
Aug 2016122925
Sep 2016101010
Oct 2016122317
Nov 20161188
Dec 20161239
Total 2016159254166
Jan 201742612
Feb 20170173
Mar 201755516
Apr 20175156
May 201735110
Jun 201731714
Jul 20172152
Aug 201732513
Sep 20172139
Oct 20171144
Nov 201752914
Dec 20172234
Total 201735300107
Jan 201804115
Feb 20182299
Mar 201823316
Apr 201834511
May 201833413
Jun 20182374
Jul 20182219
Aug 20181316
Sep 20182298
Oct 20181355
Nov 201834811
Dec 20183335
Total 201824416112
Jan 20192407
Feb 20193306
Mar 20192318
Apr 201912315
May 2019123617
Jun 20193164
Jul 201943012
Aug 201936420
Sep 201915915
Oct 201987712
Nov 201933016
Dec 20195248
Total 201947460140
Jan 202022510
Feb 20202236
Mar 20200246
Apr 20207177
May 202042610
Jun 202033812
Jul 20202169
Aug 20202148
Sep 202061414
Oct 202012513
Nov 202041912
Dec 2020102215
Total 202043263122
Jan 202152911
Feb 202134212
Mar 2021143
Total 202197526
Total220352152879
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The Journal of Neuroscience: 24 (41)
Journal of Neuroscience
Vol. 24, Issue 41
13 Oct 2004
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A Mouse Model of Classical Late-Infantile Neuronal Ceroid Lipofuscinosis Based on Targeted Disruption of the CLN2 Gene Results in a Loss of Tripeptidyl-Peptidase I Activity and Progressive Neurodegeneration
David E. Sleat, Jennifer A. Wiseman, Mukarram El-Banna, Kwi-Hye Kim, Qinwen Mao, Sandy Price, Shannon L. Macauley, Richard L. Sidman, Michael M. Shen, Qi Zhao, Marco A. Passini, Beverly L. Davidson, Gregory R. Stewart, Peter Lobel
Journal of Neuroscience 13 October 2004, 24 (41) 9117-9126; DOI: 10.1523/JNEUROSCI.2729-04.2004

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A Mouse Model of Classical Late-Infantile Neuronal Ceroid Lipofuscinosis Based on Targeted Disruption of the CLN2 Gene Results in a Loss of Tripeptidyl-Peptidase I Activity and Progressive Neurodegeneration
David E. Sleat, Jennifer A. Wiseman, Mukarram El-Banna, Kwi-Hye Kim, Qinwen Mao, Sandy Price, Shannon L. Macauley, Richard L. Sidman, Michael M. Shen, Qi Zhao, Marco A. Passini, Beverly L. Davidson, Gregory R. Stewart, Peter Lobel
Journal of Neuroscience 13 October 2004, 24 (41) 9117-9126; DOI: 10.1523/JNEUROSCI.2729-04.2004
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