If you made any changes in Pure these will be visible here soon.

Research Output

  • 6442 Citations
  • 46 h-Index
  • 90 Article
  • 4 Review article
  • 1 Chapter
2019

Analysis of brain and cerebrospinal fluid from mouse models of the three major forms of neuronal ceroid lipofuscinosis reveals changes in the lysosomal proteome

Sleat, D. E., Wiseman, J. A., El-Banna, M., Zheng, H., Zhao, C., Soherwardy, A., Moore, D. F. & Lobel, P., Jan 1 2019, In : Molecular and Cellular Proteomics. 18, 11, p. 2244-2261 18 p.

Research output: Contribution to journalArticle

2018

Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis

Nemtsova, Y., Wiseman, J. A., El-Banna, M., Lobel, P. & Sleat, D. E., Feb 2018, In : PloS one. 13, 2, e0192286.

Research output: Contribution to journalArticle

2 Scopus citations

Lysosomal enzyme tripeptidyl peptidase 1 destabilizes fibrillar Aβ by multiple endoproteolytic cleavages within the β-sheet domain

Solé-Domènech, S., Rojas, A. V., Maisuradze, G. G., Scheraga, H. A., Lobel, P. & Maxfield, F. R., Feb 13 2018, In : Proceedings of the National Academy of Sciences of the United States of America. 115, 7, p. 1493-1498 6 p.

Research output: Contribution to journalArticle

8 Scopus citations
2017

A Basic ApoE-Based Peptide Mediator to Deliver Proteins across the Blood-Brain Barrier: Long-Term Efficacy, Toxicity, and Mechanism

Meng, Y., Wiseman, J. A., Nemtsova, Y., Moore, D., Guevarra, J., Reuhl, K., Banks, W. A., Daneman, R., Sleat, D. E. & Lobel, P., Jul 5 2017, In : Molecular Therapy. 25, 7, p. 1531-1543 13 p.

Research output: Contribution to journalArticle

9 Scopus citations

Accounting for protein subcellular localization: A compartmental map of the rat liver proteome

Jadot, M., Boonen, M., Thirion, J., Wang, N., Xing, J., Zhao, C., Tannous, A., Qian, M., Zheng, H., Everett, J. K., Moore, D., Sleat, D. E. & Lobel, P., Feb 2017, In : Molecular and Cellular Proteomics. 16, 2, p. 194-212 19 p.

Research output: Contribution to journalArticle

24 Scopus citations

Chronic Enzyme Replacement to the Brain of a Late Infantile Neuronal Ceroid Lipofuscinosis Mouse Has Differential Effects on Phenotypes of Disease

Wiseman, J. A., Meng, Y., Nemtsova, Y., Matteson, P. G., Millonig, J., Moore, D., Sleat, D. E. & Lobel, P., Mar 17 2017, In : Molecular Therapy - Methods and Clinical Development. 4, p. 204-212 9 p.

Research output: Contribution to journalArticle

7 Scopus citations

Proteomic analysis of brain and cerebrospinal fluid from the three major forms of neuronal ceroid lipofuscinosis reveals potential biomarkers

Sleat, D. E., Tannous, A., Sohar, I., Wiseman, J. A., Zheng, H., Qian, M., Zhao, C., Xin, W., Barone, R., Sims, K. B., Moore, D. & Lobel, P., Oct 6 2017, In : Journal of Proteome Research. 16, 10, p. 3787-3804 18 p.

Research output: Contribution to journalArticle

6 Scopus citations

Using whole-exome sequencing to investigate the genetic bases of lysosomal storage diseases of unknown etiology

Wang, N., Zhang, Y., Gedvilaite, E., Loh, J. W., Lin, T., Liu, X., Liu, C. G., Kumar, D., Donnelly, R., Raymond, K., Schuchman, E. H., Sleat, D. E., Lobel, P. & Xing, J., Nov 2017, In : Human mutation. 38, 11, p. 1491-1499 9 p.

Research output: Contribution to journalArticle

2016

Analysis of large-scale whole exome sequencing data to determine the prevalence of genetically-distinct forms of neuronal ceroid lipofuscinosis

Sleat, D. E., Gedvilaite, E., Zhang, Y., Lobel, P. & Xing, J., Nov 30 2016, In : Gene. 593, 2, p. 284-291 8 p.

Research output: Contribution to journalArticle

13 Scopus citations
2014

Effective intravenous therapy for neurodegenerative disease with a therapeutic enzyme and a peptide that mediates delivery to the brain

Meng, Y., Sohar, I., Sleat, D. E., Richardson, J. R., Reuhl, K., Jenkins, R. B., Sarkar, G. & Lobel, P., Mar 2014, In : Molecular Therapy. 22, 3, p. 547-553 7 p.

Research output: Contribution to journalArticle

37 Scopus citations

Potential pitfalls and solutions for use of fluorescent fusion proteins to study the lysosome

Huang, L., Pike, D., Sleat, D. E., Nanda, V. & Lobel, P., Feb 21 2014, In : PloS one. 9, 2, e88893.

Research output: Contribution to journalArticle

24 Scopus citations
2013

Extending the mannose 6-phosphate glycoproteome by high resolution/accuracy mass spectrometry analysis of control and acid phosphatase 5-deficient mice

Sleat, D. E., Sun, P., Wiseman, J. A., Huang, L., El-Banna, M., Zheng, H., Moore, D. & Lobel, P., Jul 1 2013, In : Molecular and Cellular Proteomics. 12, 7, p. 1806-1817 12 p.

Research output: Contribution to journalArticle

24 Scopus citations

Tripeptidyl Peptidase I

Sohar, I., Sleat, D. E. & Lobel, P., Aug 29 2013, Handbook of Proteolytic Enzymes. Elsevier Ltd, Vol. 3. p. 3350-3356 7 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations
2012

Proteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain

Sleat, D. E., Wiseman, J. A., Sohar, I., El-Banna, M., Zheng, H., Moore, D. & Lobel, P., Dec 1 2012, In : Proteomics. 12, 23-24, p. 3499-3509 11 p.

Research output: Contribution to journalArticle

11 Scopus citations

Systemic administration of tripeptidyl peptidase i in a mouse model of late infantile neuronal ceroid lipofuscinosis: Effect of glycan modification

Meng, Y., Sohar, I., Wang, L., Sleat, D. E. & Lobel, P., Jul 6 2012, In : PloS one. 7, 7, e40509.

Research output: Contribution to journalArticle

20 Scopus citations
2011

Classification of subcellular location by comparative proteomic analysis of native and density-shifted lysosomes

Della Valle, M. C., Sleat, D. E., Zheng, H., Moore, D., Jadot, M. & Lobel, P., Apr 1 2011, In : Molecular and Cellular Proteomics. 10, 4

Research output: Contribution to journalArticle

21 Scopus citations

Intrathecal tripeptidyl-peptidase 1 reduces lysosomal storage in a canine model of late infantile neuronal ceroid lipofuscinosis

Vuillemenot, B. R., Katz, M. L., Coates, J. R., Kennedy, D., Tiger, P., Kanazono, S., Lobel, P., Sohar, I., Xu, S., Cahayag, R., Keve, S., Koren, E., Bunting, S., Tsuruda, L. S. & O'Neill, C. A., Nov 1 2011, In : Molecular Genetics and Metabolism. 104, 3, p. 325-337 13 p.

Research output: Contribution to journalArticle

46 Scopus citations

Large-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis

Xu, S., Wang, L., El-Banna, M., Sohar, I., Sleat, D. E. & Lobel, P., Oct 2011, In : Molecular Therapy. 19, 10, p. 1842-1848 7 p.

Research output: Contribution to journalArticle

39 Scopus citations

Loss of niemann-pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway

Dixit, S. S., Jadot, M., Sohar, I., Sleat, D. E., Stock, A. & Lobel, P., Aug 24 2011, In : PloS one. 6, 8, e23677.

Research output: Contribution to journalArticle

18 Scopus citations
2010

Functions of the α, β, and γsubunits of UDP-GlcNAc:Lysosomal enzyme N-acetylglucosamine-1-phosphotransferase

Qian, Y., Lee, I., Lee, W. S., Qian, M., Kudo, M., Canfield, W. M., Lobel, P. & Kornfeld, S., Jan 29 2010, In : Journal of Biological Chemistry. 285, 5, p. 3360-3370 11 p.

Research output: Contribution to journalArticle

42 Scopus citations
6 Scopus citations
2009
35 Scopus citations

Genetic modulation of apoptotic pathways fails to alter disease course in tripeptidyl-peptidase 1 deficient mice

Kim, K. H., Sleat, D. E., Bernard, O. & Lobel, P., Mar 27 2009, In : Neuroscience Letters. 453, 1, p. 27-30 4 p.

Research output: Contribution to journalArticle

7 Scopus citations

Mass spectrometry-based protein profiling to determine the cause of lysosomal storage diseases of unknown etiology

Sleat, D. E., Ding, L., Wang, S., Zhao, C., Wang, Y., Xin, W., Zheng, H., Moore, D., Sims, K. B. & Lobel, P., Dec 8 2009, In : Molecular and Cellular Proteomics. 8, 7, p. 1708-1718 11 p.

Research output: Contribution to journalArticle

36 Scopus citations

Proteomics of the lysosome

Lübke, T., Lobel, P. & Sleat, D. E., Apr 1 2009, In : Biochimica et Biophysica Acta - Molecular Cell Research. 1793, 4, p. 625-635 11 p.

Research output: Contribution to journalReview article

142 Scopus citations
2008

Acid phosphatase 5 is responsible for removing the mannose 6-phosphate recognition marker from lysosomal proteins

Sun, P., Sleat, D. E., Lecocq, M., Hayman, A. R., Jadot, M. & Lobel, P., Oct 28 2008, In : Proceedings of the National Academy of Sciences of the United States of America. 105, 43, p. 16590-16595 6 p.

Research output: Contribution to journalArticle

41 Scopus citations

A method to assess the lysosomal residence of proteins in cultured cells

Gasingirwa, M. C., Thirion, J., Costa, C., Flamion, B., Lobel, P. & Jadot, M., Mar 1 2008, In : Analytical Biochemistry. 374, 1, p. 31-40 10 p.

Research output: Contribution to journalArticle

6 Scopus citations

Degradation of fibrillar forms of Alzheimer's amyloid β-peptide by macrophages

Majumdar, A., Chung, H., Dolios, G., Wang, R., Asamoah, N., Lobel, P. & Maxfield, F. R., May 1 2008, In : Neurobiology of Aging. 29, 5, p. 707-715 9 p.

Research output: Contribution to journalArticle

54 Scopus citations

Dipeptidyl-peptidase I does not functionally compensate for the loss of tripeptidyl-peptidase I in the neurodegenerative disease late-infantile neuronal ceroid lipofuscinosis

Kim, K. H., Pham, C. T., Sleat, D. E. & Lobel, P., Oct 15 2008, In : Biochemical Journal. 415, 2, p. 225-232 8 p.

Research output: Contribution to journalArticle

2 Scopus citations

Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis

Chang, M., Cooper, J. D., Sleat, D. E., Cheng, S. H., Dodge, J. C., Passini, M. A., Lobel, P. & Davidson, B. L., Apr 2008, In : Molecular Therapy. 16, 4, p. 649-656 8 p.

Research output: Contribution to journalArticle

115 Scopus citations
55 Scopus citations

Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis

Sleat, D. E., El-Banna, M., Sohar, I., Kim, K. H., Dobrenis, K., Walkley, S. U. & Lobel, P., Jun 1 2008, In : Molecular Genetics and Metabolism. 94, 2, p. 222-233 12 p.

Research output: Contribution to journalArticle

35 Scopus citations

The mannose 6-phosphate glycoprotein proteome

Sleat, D. E., Valle, M. C. D., Zheng, H., Moore, D. & Lobel, P., Jul 1 2008, In : Journal of Proteome Research. 7, 7, p. 3010-3021 12 p.

Research output: Contribution to journalArticle

43 Scopus citations
2007

Activation of microglia acidifies lysosomes and leads to degradation of Alzheimer amyloid fibrils

Majumdar, A., Cruz, D., Asamoah, N., Buxbaum, A., Sohar, I., Lobel, P. & Maxfield, F. R., Apr 1 2007, In : Molecular biology of the cell. 18, 4, p. 1490-1496 7 p.

Research output: Contribution to journalArticle

145 Scopus citations

Do mammalian NPC1 and NPC2 play a role in intestinal cholesterol absorption?

Dixit, S. S., Sleat, D. E., Stock, A. & Lobel, P., Nov 15 2007, In : Biochemical Journal. 408, 1, p. 1-5 5 p.

Research output: Contribution to journalArticle

28 Scopus citations

Lysosomal proteomics and disease

Sleat, D. E., Jadot, M. & Lobel, P., Sep 1 2007, In : Proteomics - Clinical Applications. 1, 9, p. 1134-1146 13 p.

Research output: Contribution to journalReview article

19 Scopus citations

Structural basis of sterol binding by NPC2, a lysosomal protein deficient in Niemann-Pick type C2 disease

Xu, S., Benoff, B., Liou, H. L., Lobel, P. & Stock, A., Aug 10 2007, In : Journal of Biological Chemistry. 282, 32, p. 23525-23531 7 p.

Research output: Contribution to journalArticle

138 Scopus citations

The human urine mannose 6-phosphate glycoproteome

Sleat, D. E., Zheng, H. & Lobel, P., Mar 1 2007, In : Biochimica et Biophysica Acta - Proteins and Proteomics. 1774, 3, p. 368-372 5 p.

Research output: Contribution to journalArticle

22 Scopus citations

Timing of therapeutic intervention determines functional and survival outcomes in a mouse model of late infantile batten disease

Cabrera-Salazar, M. A., Roskelley, E. M., Bu, J., Hodges, B. L., Yew, N., Dodge, J. C., Shihabuddin, L. S., Sohar, I., Sleat, D. E., Scheule, R. K., Davidson, B. L., Cheng, S. H., Lobel, P. & Passini, M. A., Oct 1 2007, In : Molecular Therapy. 15, 10, p. 1782-1788 7 p.

Research output: Contribution to journalArticle

55 Scopus citations
2006

A frame shift mutation in canine TPP1 (the ortholog of human CLN2) in a juvenile Dachshund with neuronal ceroid lipofuscinosis

Awano, T., Katz, M. L., O'Brien, D. P., Sohar, I., Lobel, P., Coates, J. R., Khan, S., Johnson, G. C., Giger, U. & Johnson, G. S., Nov 1 2006, In : Molecular Genetics and Metabolism. 89, 3, p. 254-260 7 p.

Research output: Contribution to journalArticle

86 Scopus citations

A mutation in the cathepsin D gene (CTSD) in American Bulldogs with neuronal ceroid lipofuscinosis

Awano, T., Katz, M. L., O'Brien, D. P., Taylor, J. F., Evans, J., Khan, S., Sohar, I., Lobel, P. & Johnson, G. S., Apr 1 2006, In : Molecular Genetics and Metabolism. 87, 4, p. 341-348 8 p.

Research output: Contribution to journalArticle

97 Scopus citations

Demonstration of lysosomal localization for the mammalian ependymin-related protein using classical approaches combined with a novel density shift method

Della Valle, M. C., Sleat, D. E., Sohar, I., Wen, T., Pintar, J., Jadot, M. & Lobel, P., Nov 17 2006, In : Journal of Biological Chemistry. 281, 46, p. 35436-35445 10 p.

Research output: Contribution to journalArticle

18 Scopus citations
31 Scopus citations

Identification and validation of mannose 6-phosphate glycoproteins in human plasma reveal a wide range of lysosomal and non-lysosomal proteins

Sleat, D. E., Wang, Y., Sohar, I., Lackland, H., Li, Y., Li, H., Zheng, H. & Lobel, P., Oct 1 2006, In : Molecular and Cellular Proteomics. 5, 10, p. 1942-1956 15 p.

Research output: Contribution to journalArticle

51 Scopus citations

Identification of sites of mannose 6-phosphorylation on lysosomal proteins

Sleat, D. E., Zheng, H., Qian, M. & Lobel, P., Apr 1 2006, In : Molecular and Cellular Proteomics. 5, 4, p. 686-701 16 p.

Research output: Contribution to journalArticle

76 Scopus citations

Intracranial delivery of CLN2 reduces brain pathology in a mouse model of classical late infantile neuronal ceroid lipofuscinosis

Passini, M. A., Dodge, J. C., Bu, J., Yang, W., Zhao, Q., Sondhi, D., Hackett, N. R., Kaminsky, S. M., Mao, Q., Shihabuddin, L. S., Cheng, S. H., Sleat, D. E., Stewart, G. R., Davidson, B. L., Lobel, P. & Crystal, R. G., Feb 1 2006, In : Journal of Neuroscience. 26, 5, p. 1334-1342 9 p.

Research output: Contribution to journalArticle

95 Scopus citations

Mechanism of cholesterol transfer from the Niemann-Pick type C2 protein to model membranes supports a role in lysosomal cholesterol transport

Cheruku, S. R., Xu, Z., Dutia, R., Lobel, P. & Storch, J., Oct 20 2006, In : Journal of Biological Chemistry. 281, 42, p. 31594-31604 11 p.

Research output: Contribution to journalArticle

150 Scopus citations

NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols

Liou, H. L., Dixit, S. S., Xu, S., Tint, G. S., Stock, A. & Lobel, P., Dec 1 2006, In : Journal of Biological Chemistry. 281, 48, p. 36710-36723 14 p.

Research output: Contribution to journalArticle

81 Scopus citations
2005

The human brain mannose 6-phosphate glycoproteome: A complex mixture composed of multiple isoforms of many soluble lysosomal proteins

Sleat, D. E., Lackland, H., Wang, Y., Sohar, I., Xiao, G., Li, H. & Lobel, P., Jan 1 2005, In : Proteomics. 5, 6, p. 1520-1532 13 p.

Research output: Contribution to journalArticle

67 Scopus citations
2004

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

Sleat, D. E., Wiseman, J. A., El-Banna, M., Kim, K. H., Mao, Q., Price, S., Macauley, S. L., Sidman, R. L., Shen, M. M., Zhao, Q., Passini, M. A., Davidson, B. L., Stewart, G. R. & Lobel, P., Oct 13 2004, In : Journal of Neuroscience. 24, 41, p. 9117-9126 10 p.

Research output: Contribution to journalArticle

97 Scopus citations