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Items: 1 to 50 of 69

1.

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.

2.

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.

3.

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.

PMID:
28703315
4.

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.

5.

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.

6.

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.

7.

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.

8.

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.

9.

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.

10.

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.

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.

12.

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.

13.

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.

14.

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.

15.

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.

17.

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.

18.

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.

19.

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.

20.

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.

22.

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.

23.

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.

24.

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.

25.

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.

26.

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.

27.

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.

PMID:
21136763
28.

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.

29.

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.

30.

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.

31.

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.

32.

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.

33.

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.

34.

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.

PMID:
15789345
35.

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.

36.

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.

37.

Aminoglycoside-mediated suppression of nonsense mutations in late infantile neuronal ceroid lipofuscinosis.

Sleat DE, Sohar I, Gin RM, Lobel P.

Eur J Paediatr Neurol. 2001;5 Suppl A:57-62.

PMID:
11589009
38.

Congenital ovine neuronal ceroid lipofuscinosis--a cathepsin D deficiency with increased levels of the inactive enzyme.

Tyynelä J, Sohar I, Sleat DE, Gin RM, Donnelly RJ, Baumann M, Haltia M, Lobel P.

Eur J Paediatr Neurol. 2001;5 Suppl A:43-5.

PMID:
11589006
39.

Niemann-Pick disease type C: spectrum of HE1 mutations and genotype/phenotype correlations in the NPC2 group.

Millat G, Chikh K, Naureckiene S, Sleat DE, Fensom AH, Higaki K, Elleder M, Lobel P, Vanier MT.

Am J Hum Genet. 2001 Nov;69(5):1013-21. Epub 2001 Sep 20.

40.

Identification of HE1 as the second gene of Niemann-Pick C disease.

Naureckiene S, Sleat DE, Lackland H, Fensom A, Vanier MT, Wattiaux R, Jadot M, Lobel P.

Science. 2000 Dec 22;290(5500):2298-301.

41.

A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.

Tyynelä J, Sohar I, Sleat DE, Gin RM, Donnelly RJ, Baumann M, Haltia M, Lobel P.

EMBO J. 2000 Jun 15;19(12):2786-92.

42.

Prenatal testing for late infantile neuronal ceroid lipofuscinosis.

Berry-Kravis E, Sleat DE, Sohar I, Meyer P, Donnelly R, Lobel P.

Ann Neurol. 2000 Feb;47(2):254-7.

PMID:
10665500
44.

Subcellular localization of mannose 6-phosphate glycoproteins in rat brain.

Jadot M, Lin L, Sleat DE, Sohar I, Hsu MS, Pintar J, Dubois F, Wattiaux-De Coninck S, Wattiaux R, Lobel P.

J Biol Chem. 1999 Jul 23;274(30):21104-13.

45.

Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder.

Sleat DE, Gin RM, Sohar I, Wisniewski K, Sklower-Brooks S, Pullarkat RK, Palmer DN, Lerner TJ, Boustany RM, Uldall P, Siakotos AN, Donnelly RJ, Lobel P.

Am J Hum Genet. 1999 Jun;64(6):1511-23. Erratum in: Am J Hum Genet. 2004 Dec;75(6):1158.

46.

Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses.

Sleat DE, Sohar I, Pullarkat PS, Lobel P, Pullarkat RK.

Biochem J. 1998 Sep 15;334 ( Pt 3):547-51.

47.
48.

Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis.

Sleat DE, Donnelly RJ, Lackland H, Liu CG, Sohar I, Pullarkat RK, Lobel P.

Science. 1997 Sep 19;277(5333):1802-5.

49.

alpha-Glucosidase and N-acetylglucosamine-6-sulphatase are the major mannose-6-phosphate glycoproteins in human urine.

Sleat DE, Kraus SR, Sohar I, Lackland H, Lobel P.

Biochem J. 1997 May 15;324 ( Pt 1):33-9.

50.

Ligand binding specificities of the two mannose 6-phosphate receptors.

Sleat DE, Lobel P.

J Biol Chem. 1997 Jan 10;272(2):731-8.

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