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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 DE, Wiseman JA, El-Banna M, Zheng H, Zhao C, Soherwardy A, Moore DF, Lobel P.

Mol Cell Proteomics. 2019 Sep 9. pii: mcp.RA119.001587. doi: 10.1074/mcp.RA119.001587. [Epub ahead of print]


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

Nemtsova Y, Wiseman JA, El-Banna M, Lobel P, Sleat DE.

PLoS One. 2018 Feb 6;13(2):e0192286. doi: 10.1371/journal.pone.0192286. eCollection 2018.


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

Solé-Domènech S, Rojas AV, Maisuradze GG, Scheraga HA, Lobel P, Maxfield FR.

Proc Natl Acad Sci U S A. 2018 Feb 13;115(7):1493-1498. doi: 10.1073/pnas.1719808115. Epub 2018 Jan 29.


Proteomic Analysis of Brain and Cerebrospinal Fluid from the Three Major Forms of Neuronal Ceroid Lipofuscinosis Reveals Potential Biomarkers.

Sleat DE, Tannous A, Sohar I, Wiseman JA, Zheng H, Qian M, Zhao C, Xin W, Barone R, Sims KB, Moore DF, Lobel P.

J Proteome Res. 2017 Oct 6;16(10):3787-3804. doi: 10.1021/acs.jproteome.7b00460. Epub 2017 Aug 28.


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

Wang N, Zhang Y, Gedvilaite E, Loh JW, Lin T, Liu X, Liu CG, Kumar D, Donnelly R, Raymond K, Schuchman EH, Sleat DE, Lobel P, Xing J.

Hum Mutat. 2017 Nov;38(11):1491-1499. doi: 10.1002/humu.23291. Epub 2017 Jul 25.


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

Meng Y, Wiseman JA, Nemtsova Y, Moore DF, Guevarra J, Reuhl K, Banks WA, Daneman R, Sleat DE, Lobel P.

Mol Ther. 2017 Jul 5;25(7):1531-1543. doi: 10.1016/j.ymthe.2017.03.037. Epub 2017 Apr 26.


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

Wiseman JA, Meng Y, Nemtsova Y, Matteson PG, Millonig JH, Moore DF, Sleat DE, Lobel P.

Mol Ther Methods Clin Dev. 2017 Feb 13;4:204-212. doi: 10.1016/j.omtm.2017.01.004. eCollection 2017 Mar 17.


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 JK, Moore DF, Sleat DE, Lobel P.

Mol Cell Proteomics. 2017 Feb;16(2):194-212. doi: 10.1074/mcp.M116.064527. Epub 2016 Dec 6.


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

Sleat DE, Gedvilaite E, Zhang Y, Lobel P, Xing J.

Gene. 2016 Nov 30;593(2):284-91. doi: 10.1016/j.gene.2016.08.031. Epub 2016 Aug 20.


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

Huang L, Pike D, Sleat DE, Nanda V, Lobel P.

PLoS One. 2014 Feb 21;9(2):e88893. doi: 10.1371/journal.pone.0088893. eCollection 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 DE, Richardson JR, Reuhl KR, Jenkins RB, Sarkar G, Lobel P.

Mol Ther. 2014 Mar;22(3):547-553. doi: 10.1038/mt.2013.267. Epub 2013 Dec 26.


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

Sleat DE, Sun P, Wiseman JA, Huang L, El-Banna M, Zheng H, Moore DF, Lobel P.

Mol Cell Proteomics. 2013 Jul;12(7):1806-17. doi: 10.1074/mcp.M112.026179. Epub 2013 Mar 11.


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

Sleat DE, Wiseman JA, Sohar I, El-Banna M, Zheng H, Moore DF, Lobel P.

Proteomics. 2012 Dec;12(23-24):3499-509. doi: 10.1002/pmic.201200205. Epub 2012 Nov 22.


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 DE, Lobel P.

PLoS One. 2012;7(7):e40509. doi: 10.1371/journal.pone.0040509. Epub 2012 Jul 6.


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

Dixit SS, Jadot M, Sohar I, Sleat DE, Stock AM, Lobel P.

PLoS One. 2011;6(8):e23677. doi: 10.1371/journal.pone.0023677. Epub 2011 Aug 24.


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

Vuillemenot BR, Katz ML, Coates JR, Kennedy D, Tiger P, Kanazono S, Lobel P, Sohar I, Xu S, Cahayag R, Keve S, Koren E, Bunting S, Tsuruda LS, O'Neill CA.

Mol Genet Metab. 2011 Nov;104(3):325-37. doi: 10.1016/j.ymgme.2011.06.018. Epub 2011 Jun 30.


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 DE, Lobel P.

Mol Ther. 2011 Oct;19(10):1842-8. doi: 10.1038/mt.2011.130. Epub 2011 Jul 5.


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

Della Valle MC, Sleat DE, Zheng H, Moore DF, Jadot M, Lobel P.

Mol Cell Proteomics. 2011 Apr;10(4):M110.006403. doi: 10.1074/mcp.M110.006403. Epub 2011 Jan 20.


Functions of the alpha, beta, and gamma subunits of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase.

Qian Y, Lee I, Lee WS, Qian M, Kudo M, Canfield WM, Lobel P, Kornfeld S.

J Biol Chem. 2010 Jan 29;285(5):3360-70. doi: 10.1074/jbc.M109.068650. Epub 2009 Dec 2.


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

Kim KH, Sleat DE, Bernard O, Lobel P.

Neurosci Lett. 2009 Mar 27;453(1):27-30. doi: 10.1016/j.neulet.2009.01.072. Epub 2009 Feb 4.


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

Sleat DE, Ding L, Wang S, Zhao C, Wang Y, Xin W, Zheng H, Moore DF, Sims KB, Lobel P.

Mol Cell Proteomics. 2009 Jul;8(7):1708-18. doi: 10.1074/mcp.M900122-MCP200. Epub 2009 Apr 20.


Crystal structure and autoactivation pathway of the precursor form of human tripeptidyl-peptidase 1, the enzyme deficient in late infantile ceroid lipofuscinosis.

Guhaniyogi J, Sohar I, Das K, Stock AM, Lobel P.

J Biol Chem. 2009 Feb 6;284(6):3985-97. doi: 10.1074/jbc.M806943200. Epub 2008 Nov 26.


Proteomics of the lysosome.

Lübke T, Lobel P, Sleat DE.

Biochim Biophys Acta. 2009 Apr;1793(4):625-35. doi: 10.1016/j.bbamcr.2008.09.018. Epub 2008 Oct 15. Review.


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

Sun P, Sleat DE, Lecocq M, Hayman AR, Jadot M, Lobel P.

Proc Natl Acad Sci U S A. 2008 Oct 28;105(43):16590-5. doi: 10.1073/pnas.0807472105. Epub 2008 Oct 21.


The mannose 6-phosphate glycoprotein proteome.

Sleat DE, Della Valle MC, Zheng H, Moore DF, Lobel P.

J Proteome Res. 2008 Jul;7(7):3010-21. doi: 10.1021/pr800135v. Epub 2008 May 29.


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

Chang M, Cooper JD, Sleat DE, Cheng SH, Dodge JC, Passini MA, Lobel P, Davidson BL.

Mol Ther. 2008 Apr;16(4):649-56. doi: 10.1038/mt.2008.9. Epub 2008 Feb 12.


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

Sleat DE, El-Banna M, Sohar I, Kim KH, Dobrenis K, Walkley SU, Lobel P.

Mol Genet Metab. 2008 Jun;94(2):222-33. doi: 10.1016/j.ymgme.2008.01.014. Epub 2008 Mar 17.


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

Gasingirwa MC, Thirion J, Costa C, Flamion B, Lobel P, Jadot M.

Anal Biochem. 2008 Mar 1;374(1):31-40. Epub 2007 Nov 22.


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

Dixit SS, Sleat DE, Stock AM, Lobel P.

Biochem J. 2007 Nov 15;408(1):1-5.


Proteomics analysis of serum from mutant mice reveals lysosomal proteins selectively transported by each of the two mannose 6-phosphate receptors.

Qian M, Sleat DE, Zheng H, Moore D, Lobel P.

Mol Cell Proteomics. 2008 Jan;7(1):58-70. Epub 2007 Sep 11.


Lysosomal proteomics and disease.

Sleat DE, Jadot M, Lobel P.

Proteomics Clin Appl. 2007 Sep;1(9):1134-46. doi: 10.1002/prca.200700250. Epub 2007 Aug 17.


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

Cabrera-Salazar MA, Roskelley EM, Bu J, Hodges BL, Yew N, Dodge JC, Shihabuddin LS, Sohar I, Sleat DE, Scheule RK, Davidson BL, Cheng SH, Lobel P, Passini MA.

Mol Ther. 2007 Oct;15(10):1782-8. Epub 2007 Jul 17.


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

Xu S, Benoff B, Liou HL, Lobel P, Stock AM.

J Biol Chem. 2007 Aug 10;282(32):23525-31. Epub 2007 Jun 14.


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 FR.

Mol Biol Cell. 2007 Apr;18(4):1490-6. Epub 2007 Feb 21.


The human urine mannose 6-phosphate glycoproteome.

Sleat DE, Zheng H, Lobel P.

Biochim Biophys Acta. 2007 Mar;1774(3):368-72. Epub 2006 Dec 20.


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

Majumdar A, Chung H, Dolios G, Wang R, Asamoah N, Lobel P, Maxfield FR.

Neurobiol Aging. 2008 May;29(5):707-15. Epub 2007 Jan 11.


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

Liou HL, Dixit SS, Xu S, Tint GS, Stock AM, Lobel P.

J Biol Chem. 2006 Dec 1;281(48):36710-23. Epub 2006 Oct 2.


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

Della Valle MC, Sleat DE, Sohar I, Wen T, Pintar JE, Jadot M, Lobel P.

J Biol Chem. 2006 Nov 17;281(46):35436-45. Epub 2006 Sep 5.


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

Sleat DE, Wang Y, Sohar I, Lackland H, Li Y, Li H, Zheng H, Lobel P.

Mol Cell Proteomics. 2006 Oct;5(10):1942-56. Epub 2006 May 17.


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

Awano T, Katz ML, O'Brien DP, Sohar I, Lobel P, Coates JR, Khan S, Johnson GC, Giger U, Johnson GS.

Mol Genet Metab. 2006 Nov;89(3):254-60. Epub 2006 Apr 18.


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

Cheruku SR, Xu Z, Dutia R, Lobel P, Storch J.

J Biol Chem. 2006 Oct 20;281(42):31594-604. Epub 2006 Apr 10.


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

Passini MA, Dodge JC, Bu J, Yang W, Zhao Q, Sondhi D, Hackett NR, Kaminsky SM, Mao Q, Shihabuddin LS, Cheng SH, Sleat DE, Stewart GR, Davidson BL, Lobel P, Crystal RG.

J Neurosci. 2006 Feb 1;26(5):1334-42.


Identification of sites of mannose 6-phosphorylation on lysosomal proteins.

Sleat DE, Zheng H, Qian M, Lobel P.

Mol Cell Proteomics. 2006 Apr;5(4):686-701. Epub 2006 Jan 5.


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

Awano T, Katz ML, O'Brien DP, Taylor JF, Evans J, Khan S, Sohar I, Lobel P, Johnson GS.

Mol Genet Metab. 2006 Apr;87(4):341-8. Epub 2006 Jan 4.


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

Sleat DE, Lackland H, Wang Y, Sohar I, Xiao G, Li H, Lobel P.

Proteomics. 2005 Apr;5(6):1520-32. Erratum in: Proteomics. 2005 May;5(8):2272.


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 DE, Wiseman JA, El-Banna M, Kim KH, Mao Q, Price S, Macauley SL, Sidman RL, Shen MM, Zhao Q, Passini MA, Davidson BL, Stewart GR, Lobel P.

J Neurosci. 2004 Oct 13;24(41):9117-26.


Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.

Sleat DE, Wiseman JA, El-Banna M, Price SM, Verot L, Shen MM, Tint GS, Vanier MT, Walkley SU, Lobel P.

Proc Natl Acad Sci U S A. 2004 Apr 20;101(16):5886-91. Epub 2004 Apr 7.

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