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David Sleat

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David E Sleat; Istvan Sohar; Jennifer A Wiseman; Haiyan Zheng; Mukarram El-Banna; Dirk F Moore; Peter Lobel
Proteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain.
Yu Meng; David E Sleat; Istvan Sohar; Lingling Wang; Peter Lobel
Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification.
Su Xu; Mukarram El-Banna; David E Sleat; Istvan Sohar; Lingling Wang; Peter Lobel
Large-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis.

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All Publications

2012: David E Sleat; Istvan Sohar; Jennifer A Wiseman; Haiyan Zheng; Mukarram El-Banna; Dirk F Moore; Peter Lobel
Proteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain.
Proteomics 2012;12(23-24):3499-509.
2012: Yu Meng; David E Sleat; Istvan Sohar; Lingling Wang; Peter Lobel
Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification.
PloS one 2012;7(7):e40509.
2011: Su Xu; Mukarram El-Banna; David E Sleat; Istvan Sohar; Lingling Wang; Peter Lobel
Large-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis.
Molecular therapy : the journal of the American Society of Gene Therapy 2011;19(10):1842-8.
2011: Maria Cecilia Della Valle; David E Sleat; Haiyan Zheng; Dirk F Moore; Michel Jadot; Peter Lobel
Classification of subcellular location by comparative proteomic analysis of native and density-shifted lysosomes.
Molecular & cellular proteomics : MCP 2011;10(4):M110.006403.
2011: Sayali S Dixit; Michel Jadot; David E Sleat; Istvan Sohar; Ann M Stock; Peter Lobel
Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway.
PloS one 2011;6(8):e23677.
2010: Su Xu; David E Sleat; Michel Jadot; Peter Lobel
Glial fibrillary acidic protein is elevated in the lysosomal storage disease classical late-infantile neuronal ceroid lipofuscinosis, but is not a component of the storage material.
The Biochemical journal 2010;428(3):355-62.
2009: David E Sleat; Lin Ding; Shudan Wang; Caifeng Zhao; Yanhong Wang; Winnie Xin; Haiyan Zheng; Dirk Moore; Katherine B Sims; Peter Lobel
Mass spectrometry-based protein profiling to determine the cause of lysosomal storage diseases of unknown etiology.
Molecular & cellular proteomics : MCP 2009;8(7):1708-18.
2009: Torben Lübke; Peter Lobel; David E Sleat
Proteomics of the lysosome.
Biochimica et biophysica acta 2009;1793(4):625-35.
2009: Kwi-Hye Kim; David E Sleat; Ora Bernard; Peter Lobel
Genetic modulation of apoptotic pathways fails to alter disease course in tripeptidyl-peptidase 1 deficient mice.
Neuroscience letters 2009;453(1):27-30.
2008: Pengling Sun; David E Sleat; Michèle Lecocq; Alison R Hayman; Michel Jadot; Peter Lobel
Acid phosphatase 5 is responsible for removing the mannose 6-phosphate recognition marker from lysosomal proteins.
Proceedings of the National Academy of Sciences of the United States of America 2008;105(43):16590-5.
2008: Kwi-Hye Kim; Christine T Pham; David E Sleat; Peter Lobel
Dipeptidyl-peptidase I does not functionally compensate for the loss of tripeptidyl-peptidase I in the neurodegenerative disease late-infantile neuronal ceroid lipofuscinosis.
The Biochemical journal 2008;415(2):225-32.
2008: David E Sleat; Maria Cecilia Della Valle; Haiyan Zheng; Dirk Moore; Peter Lobel
The mannose 6-phosphate glycoprotein proteome.
Journal of proteome research 2008;7(7):3010-21.
2008: David E Sleat; Mukarram El-Banna; Istvan Sohar; Kwi-Hye Kim; Kostantin Dobrenis; Steven U Walkley; Peter Lobel
Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis.
Molecular genetics and metabolism 2008;94(2):222-33.
2008: Michael Chang; Jonathan D Cooper; David E Sleat; Seng Cheng; James C Dodge; Marco A Passini; Peter Lobel; Beverly L Davidson
Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis.
Molecular therapy : the journal of the American Society of Gene Therapy 2008;16(4):649-56.
2008: Meiqian Qian; David E Sleat; Haiyan Zheng; Dirk Moore; Peter Lobel
Proteomics analysis of serum from mutant mice reveals lysosomal proteins selectively transported by each of the two mannose 6-phosphate receptors.
Molecular & cellular proteomics : MCP 2008;7(1):58-70.
2007: Sayali S Dixit; David E Sleat; Ann M Stock; Peter Lobel
Do mammalian NPC1 and NPC2 play a role in intestinal cholesterol absorption?
The Biochemical journal 2007;408(1):1-5.
2007: Mario A Cabrera-Salazar; Eric M Roskelley; Jie Bu; Bradley L Hodges; Nelson S Yew; James C Dodge; Lamya S Shihabuddin; Istvan Sohar; David E Sleat; Ronald K Scheule; Beverly L Davidson; Seng Cheng; Peter Lobel; Marco A Passini
Timing of therapeutic intervention determines functional and survival outcomes in a mouse model of late infantile batten disease.
Molecular therapy : the journal of the American Society of Gene Therapy 2007;15(10):1782-8.
2007: David E Sleat; Michel Jadot; Peter Lobel
Lysosomal proteomics and disease.
Proteomics. Clinical applications 2007;1(9):1134-46.
2007: David E Sleat; Haiyan Zheng; Peter Lobel
The human urine mannose 6-phosphate glycoproteome.
Biochimica et biophysica acta 2007;1774(3):368-72.
2006: Maria Cecilia Della Valle; David E Sleat; Istvan Sohar; Ting Wen; John E Pintar; Michel Jadot; Peter Lobel
Demonstration of lysosomal localization for the mammalian ependymin-related protein using classical approaches combined with a novel density shift method.
The Journal of biological chemistry 2006;281(46):35436-45.
2006: David E Sleat; Yanhong Wang; Istvan Sohar; Henry Lackland; Yan Li; Hong Li; Haiyan Zheng; Peter Lobel
Identification and validation of mannose 6-phosphate glycoproteins in human plasma reveal a wide range of lysosomal and non-lysosomal proteins.
Molecular & cellular proteomics : MCP 2006;5(10):1942-56.
2006: David E Sleat; Haiyan Zheng; Meiqian Qian; Peter Lobel
Identification of sites of mannose 6-phosphorylation on lysosomal proteins.
Molecular & cellular proteomics : MCP 2006;5(4):686-701.
2006: Marco A Passini; James C Dodge; Jie Bu; Wendy Yang; Qi Zhao; Dolan Sondhi; Neil R Hackett; Stephen M Kaminsky; Qinwen Mao; Lamya S Shihabuddin; Seng Cheng; David E Sleat; Gregory R Stewart; Beverly L Davidson; Peter Lobel; Helen-Ann Brown Epstein
Intracranial delivery of CLN2 reduces brain pathology in a mouse model of classical late infantile neuronal ceroid lipofuscinosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2006;26(5):1334-42.
2005: David E Sleat; Henry Lackland; Yanhong Wang; Istvan Sohar; Gang Xiao; Hong Li; Peter Lobel
The human brain mannose 6-phosphate glycoproteome: a complex mixture composed of multiple isoforms of many soluble lysosomal proteins.
Proteomics 2005;5(6):1520-32.
2004: 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
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.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2004;24(41):9117-26.
2004: David E Sleat; Jennifer A Wiseman; Mukarram El-Banna; Sandy M Price; Lucie Verot; Michael M Shen; Stephen G Tint; marie T vanier; Steven U Walkley; Peter Lobel
Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(16):5886-91.
2001: Gilles Millat; Karim Chikh; Saule Naureckiene; David E Sleat; AH Fensom; Katsumi Higaki; M Elleder; Peter Lobel; marie T vanier
Niemann-Pick disease type C: spectrum of HE1 mutations and genotype/phenotype correlations in the NPC2 group.
American journal of human genetics 2001;69(5):1013-21.
2001: David E Sleat; Istvan Sohar; RM Gin; Peter Lobel
Aminoglycoside-mediated suppression of nonsense mutations in late infantile neuronal ceroid lipofuscinosis.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 2001;5 Suppl A():57-62.
2001: Jaana Tyynelä; Istvan Sohar; David E Sleat; RM Gin; RJ Donnelly; M Baumann; Matti Haltia; Peter Lobel
Congenital ovine neuronal ceroid lipofuscinosis--a cathepsin D deficiency with increased levels of the inactive enzyme.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society 2001;5 Suppl A():43-5.
2000: Saule Naureckiene; David E Sleat; Henry Lackland; AH Fensom; marie T vanier; Robert Wattiaux; Michel Jadot; Peter Lobel
Identification of HE1 as the second gene of Niemann-Pick C disease.
Science (New York, N.Y.) 2000;290(5500):2298-301.
2000: Jaana Tyynelä; Istvan Sohar; David E Sleat; RM Gin; RJ Donnelly; M Baumann; Matti Haltia; Peter Lobel
A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.
The EMBO journal 2000;19(12):2786-92.
2000: Elizabeth M Berry-Kravis; David E Sleat; Istvan Sohar; P Meyer; R Donnelly; Peter Lobel
Prenatal testing for late infantile neuronal ceroid lipofuscinosis.
Annals of neurology 2000;47(2):254-7.
1999: Istvan Sohar; David E Sleat; Michel Jadot; Peter Lobel
Biochemical characterization of a lysosomal protease deficient in classical late infantile neuronal ceroid lipofuscinosis (LINCL) and development of an enzyme-based assay for diagnosis and exclusion of LINCL in human specimens and animal models.
Journal of neurochemistry 1999;73(2):700-11.
1999: Michel Jadot; L Lin; David E Sleat; Istvan Sohar; Ming-Sing Hsu; John E Pintar; F Dubois; Simone Wattiaux-De Coninck; Robert Wattiaux; Peter Lobel
Subcellular localization of mannose 6-phosphate glycoproteins in rat brain.
The Journal of biological chemistry 1999;274(30):21104-13.
1999: David E Sleat; RM Gin; Istvan Sohar; K Wisniewski; Susan Sklower Brooks; Raju K Pullarkat; D N Palmer; Terry J Lerner; Rose-Mary N Boustany; P Uldall; A N Siakotos; RJ Donnelly; Peter Lobel
Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder.
American journal of human genetics 1999;64(6):1511-23.
1998: David E Sleat; Istvan Sohar; P S Pullarkat; Peter Lobel; Raju K Pullarkat
Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses.
The Biochemical journal 1998;334 ( Pt 3)():547-51.
1998: Chang-gong liu; David E Sleat; RJ Donnelly; Peter Lobel
Structural organization and sequence of CLN2, the defective gene in classical late infantile neuronal ceroid lipofuscinosis.
Genomics 1998;50(2):206-12.
1998: Istvan Sohar; David E Sleat; C Gong Liu; T Ludwig; Peter Lobel
Mouse mutants lacking the cation-independent mannose 6-phosphate/insulin-like growth factor II receptor are impaired in lysosomal enzyme transport: comparison of cation-independent and cation-dependent mannose 6-phosphate receptor-deficient mice.
The Biochemical journal 1998;330 ( Pt 2)():903-8.
1997: David E Sleat; RJ Donnelly; Henry Lackland; Chang-gong liu; Istvan Sohar; Raju K Pullarkat; Peter Lobel
Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis.
Science (New York, N.Y.) 1997;277(5333):1802-5.
1997: David E Sleat; Stephen Kraus; Istvan Sohar; Henry Lackland; Peter Lobel
alpha-Glucosidase and N-acetylglucosamine-6-sulphatase are the major mannose-6-phosphate glycoproteins in human urine.
The Biochemical journal 1997;324 ( Pt 1)():33-9.
1997: David E Sleat; Peter Lobel
Ligand binding specificities of the two mannose 6-phosphate receptors.
The Journal of biological chemistry 1997;272(2):731-8.
1996: David E Sleat; Istvan Sohar; Henry Lackland; J Majercak; Peter Lobel
Rat brain contains high levels of mannose-6-phosphorylated glycoproteins including lysosomal enzymes and palmitoyl-protein thioesterase, an enzyme implicated in infantile neuronal lipofuscinosis.
The Journal of biological chemistry 1996;271(32):19191-8.
1995: David E Sleat; T L Chen; K Raska; Peter Lobel
Increased levels of glycoproteins containing mannose 6-phosphate in human breast carcinomas.
Cancer research 1995;55(15):3424-30.