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
Filter
Article
1983

The arrangement and functions of the chains of the acetylcholine receptor of Torpedo electric tissue.

Karlin, A., Cox, R., Kaldany, R. R., Lobel, P. & Holtzman, E., Dec 1 1983, In : Cold Spring Harbor symposia on quantitative biology. 48 Pt 1, p. 1-8 8 p.

Research output: Contribution to journalArticle

17 Scopus citations

The arrangement of the subunits of the acetylcholine receptor of Torpedo californica

Karlin, A., Holtzman, E., Yodh, N., Lobel, P., Wall, J. & Hainfeld, J., Aug 11 1983, In : Journal of Biological Chemistry. 258, 11, p. 6678-6681 4 p.

Research output: Contribution to journalArticle

66 Scopus citations
1985

Binding of a curarimimetic toxin from cobra venom to the nicotinic acetylcholine receptor. Interactions of six biotinyltoxin derivatives with receptor and avidin

Lobel, P., Kao, P. N., Birken, S. & Karlin, A., Dec 1 1985, In : Journal of Biological Chemistry. 260, 19, p. 10605-10612 8 p.

Research output: Contribution to journalArticle

27 Scopus citations
1986

Functional Domains of the Nicotinic Acetylcholine Receptor

KARLIN, ARTHUR., COX, ROBERT. N., DIPAOLA, MARIO., HOLTZMAN, ERIC., KAO, PETER. N., Lobel, P., WANG, LI. & YODH, NIYATI., Jan 1 1986, In : Annals of the New York Academy of Sciences. 463, 1, p. 53-69 17 p.

Research output: Contribution to journalArticle

8 Scopus citations
1987

46 kd mannose 6-phosphate receptor: Cloning, expression, and homology to the 215 kd mannose 6-phosphate receptor

Dahms, N. M., Lobel, P., Breitmeyer, J., Chirgwin, J. M. & Kornfeld, S., Jul 17 1987, In : Cell. 50, 2, p. 181-192 12 p.

Research output: Contribution to journalArticle

95 Scopus citations

Cloning of the bovine 215-kDa cation-independent mannose 6-phosphate receptor

Lobel, P., Dahms, D. M., Breitmeyer, J., Chirgwin, J. M. & Kornfeld, S., Jul 21 1987, In : Proceedings of the National Academy of Sciences of the United States of America. 84, 8, p. 2233-2237 5 p.

Research output: Contribution to journalArticle

83 Scopus citations
1988

Cloning and sequence analysis of the cation-independent mannose 6-phosphate receptor

Lobel, P., Dahms, N. M. & Kornfeld, S., Jan 1 1988, In : Journal of Biological Chemistry. 263, 5, p. 2563-2570 8 p.

Research output: Contribution to journalArticle

138 Scopus citations
1989

Mutations in the cytoplasmic domain of the 275 kd mannose 6-phosphate receptor differentially alter lysosomal enzyme sorting and endocytosis

Lobel, P., Fujimoto, K., Ye, R. D., Griffiths, G. & Kornfeld, S., Jun 2 1989, In : Cell. 57, 5, p. 787-796 10 p.

Research output: Contribution to journalArticle

162 Scopus citations
1992

Gene and pseudogene of the mouse cation-dependent mannose 6-phosphate receptor. Genomic organization, expression, and chromosomal localization

Ludwig, T., Ruther, U., Metzger, R., Copeland, N. G., Jenkins, N. A., Lobel, P. & Hoflack, B., Jan 1 1992, In : Journal of Biological Chemistry. 267, 17, p. 12211-12219 9 p.

Research output: Contribution to journalArticle

23 Scopus citations
1993

An assay to detect glycoproteins that contain mannose 6-phosphate

Valenzano, K. J., Kallay, L. M. & Lobel, P., Feb 15 1993, In : Analytical Biochemistry. 209, 1, p. 156-162 7 p.

Research output: Contribution to journalArticle

41 Scopus citations
79 Scopus citations

Targeted disruption of the mouse cation-dependent mannose 6-phosphate receptor results in partial missorting of multiple lysosomal enzymes

Ludwig, T., Ovitt, C. E., Bauer, U., Hollinshead, M., Remmler, J., Lobel, P., Ruther, U. & Hoflack, B., Jan 1 1993, In : EMBO Journal. 12, 13, p. 5225-5235 11 p.

Research output: Contribution to journalArticle

62 Scopus citations
1994

A method to increase efficiency and minimize anomalous electrophoretic transfer in protein blotting

Bhavsar, J. H., Remmler, J. & Lobel, P., Sep 1994, In : Analytical Biochemistry. 221, 2, p. 234-242 9 p.

Research output: Contribution to journalArticle

15 Scopus citations

Cloning and sequencing of cDNAs encoding the full-length mouse mannose 6-phosphate/insulin-like growth factor II receptor

Ludwig, T., Tenscher, K., Remmler, J., Hoflack, B. & Lobel, P., May 16 1994, In : Gene. 142, 2, p. 311-312 2 p.

Research output: Contribution to journalArticle

14 Scopus citations

Differential sorting of lysosomal enzymes in mannose 6-phosphate receptor-deficient fibroblasts

Ludwig, T., Munier-Lehmann, H., Bauer, U., Hollinshead, M., Ovitt, C., Lobel, P. & Hoflack, B., Aug 22 1994, In : EMBO Journal. 13, 15, p. 3430-3437 8 p.

Research output: Contribution to journalArticle

123 Scopus citations
1995

Increased Levels of Glycoproteins Containing Mannose 6-Phosphate in Human Breast Carcinomas

Sleat, D. E., Chen, T. L., Raska, K. & Lobel, P., Aug 1 1995, In : Cancer Research. 55, 15, p. 3424-3430 7 p.

Research output: Contribution to journalArticle

30 Scopus citations
47 Scopus citations
1996
94 Scopus citations

Re-expression of the mannose 6-phosphate receptors in receptor-deficient fibroblasts: Complementaey function of the two mannose 6-phosphate receptors in lysosomal enzyme targeting

Munier-Lehmann, H., Mauxion, F., Bauer, U., Lobel, P. & Hoflack, B., Jan 1 1996, In : Journal of Biological Chemistry. 271, 25, p. 15166-15174 9 p.

Research output: Contribution to journalArticle

50 Scopus citations
1997

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

Sleat, D. E., Donnelly, R., Lackland, H., Liu, C. G., Sohar, I., Pullarkat, R. K. & Lobel, P., Sep 19 1997, In : Science. 277, 5333, p. 1802-1805 4 p.

Research output: Contribution to journalArticle

462 Scopus citations

Biophysical and biological properties of naturally occurring high molecular weight insulin-like growth factor II variants

Valenzano, K. J., Heath-Monnig, E., Tollefsen, S. E., Lake, M. & Lobel, P., Feb 21 1997, In : Journal of Biological Chemistry. 272, 8, p. 4804-4813 10 p.

Research output: Contribution to journalArticle

26 Scopus citations

Ligand binding specificities of the two mannose 6-phosphate receptors

Sleat, D. E. & Lobel, P., Jan 25 1997, In : Journal of Biological Chemistry. 272, 2, p. 731-738 8 p.

Research output: Contribution to journalArticle

48 Scopus citations
70 Scopus citations

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

Sleat, D. E., Kraus, S. R., Sohar, I., Lackland, H. & Lobel, P., May 15 1997, In : Biochemical Journal. 324, 1, p. 33-39 7 p.

Research output: Contribution to journalArticle

12 Scopus citations
1998

Azurophilic granules of human neutrophilic leukocytes are deficient in lysosome-associated membrane proteins but retain the mannose 6-phosphate recognition marker

Cieutat, A. M., Lobel, P., August, J. T., Kjeldsen, L., Sengeløv, H., Borregaard, N. & Bainton, D. F., Feb 1 1998, In : Blood. 91, 3, p. 1044-1058 15 p.

Research output: Contribution to journalArticle

65 Scopus citations
47 Scopus citations

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

Sleat, D. E., Sohar, I., Pullarkat, P. S., Lobel, P. & Pullarkat, R. K., Sep 15 1998, In : Biochemical Journal. 334, 3, p. 547-551 5 p.

Research output: Contribution to journalArticle

41 Scopus citations

Structural organization and sequence of CLN2, the defective gene in classical late infantile neuronal ceroid lipofuscinosis

Liu, C. G., Sleat, D. E., Donnelly, R. & Lobel, P., Jun 1 1998, In : Genomics. 50, 2, p. 206-212 7 p.

Research output: Contribution to journalArticle

55 Scopus citations
1999
72 Scopus citations

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

Sleat, D. E., Gin, R. M., Sohar, I., Wisniewski, K., Brooks, S., Pullarkat, R. K., Palmer, D. N., Lerner, T. J., Boustany, R. M., Uldall, P., Siakotos, A. N., Donnelly, R. & Lobel, P., Jan 1 1999, In : American Journal of Human Genetics. 64, 6, p. 1511-1523 13 p.

Research output: Contribution to journalArticle

124 Scopus citations

Subcellular localization of mannose 6-phosphate glycoproteins in rat brain

Jadot, M., Lin, L., Sleat, D. E., Sohar, I., Hsu, M. S., Pintar, J., Dubois, F., Wattiaux-de Coninck, S., Wattiaux, R. & Lobel, P., Jul 23 1999, In : Journal of Biological Chemistry. 274, 30, p. 21104-21113 10 p.

Research output: Contribution to journalArticle

20 Scopus citations
2000

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

Tyynelä, J., Sohar, I., Sleat, D. E., Gin, R. M., Donnelly, R., Baumann, M., Haltia, M. & Lobel, P., Jun 15 2000, In : EMBO Journal. 19, 12, p. 2786-2792 7 p.

Research output: Contribution to journalArticle

189 Scopus citations
47 Scopus citations

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

Naureckiene, S., Sleat, D. E., Lacklan, H., Fensom, A., Vanier, M. T., Wattiaux, R., Jadot, M. & Lobel, P., Dec 22 2000, In : Science. 290, 5500, p. 2298-2301 4 p.

Research output: Contribution to journalArticle

612 Scopus citations

Prenatal testing for late infantile neuronal ceroid lipofuscinosis

Berry-Kravis, E., Sleat, D. E., Sohar, I., Meyer, P., Donnelly, R. & Lobel, P., Feb 14 2000, In : Annals of Neurology. 47, 2, p. 254-257 4 p.

Research output: Contribution to journalArticle

17 Scopus citations
2001

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

Sleat, D. E., Sohar, I., Gin, R. M. & Lobel, P., Jan 1 2001, In : European Journal of Paediatric Neurology. 5, SUPPL. A, p. 57-62 6 p.

Research output: Contribution to journalArticle

53 Scopus citations

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

Tyynelä, J., Sohar, I., Sleat, D. E., Gin, R. M., Donnelly, R., Baumann, M., Haltia, M. & Lobel, P., Jan 1 2001, In : European Journal of Paediatric Neurology. 5, p. 43-45 3 p., 05800091.

Research output: Contribution to journalArticle

23 Scopus citations
13 Scopus citations

Niemann-pick disease type C: Spectrum of HE1 mutations and genotype/phenotype correlations in the NPC2 group

Millat, G., Chikh, K., Naureckiene, S., Sleat, D. E., Fensom, A. H., Higaki, K., Elleder, M., Lobel, P. & Vanier, M. T., Jan 1 2001, In : American Journal of Human Genetics. 69, 5, p. 1013-1021 9 p.

Research output: Contribution to journalArticle

122 Scopus citations
65 Scopus citations

The human CLN2 protein/tripeptidyl-peptidase I is a serine protease that autoactivates at acidic pH

Lin, L., Sohar, I., Lackland, H. & Lobel, P., Jan 19 2001, In : Journal of Biological Chemistry. 276, 3, p. 2249-2255 7 p.

Research output: Contribution to journalArticle

101 Scopus citations
2003

Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease

Friedland, N., Liou, H. L., Lobel, P. & Stock, A., Mar 4 2003, In : Proceedings of the National Academy of Sciences of the United States of America. 100, 5, p. 2512-2517 6 p.

Research output: Contribution to journalArticle

227 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

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

Sleat, D. E., Wiseman, J. A., El-Banna, M., Price, S. M., Verot, L., Shen, M. M., Tint, G. S., Vanier, M. T., Walkley, S. U. & Lobel, P., Apr 20 2004, In : Proceedings of the National Academy of Sciences of the United States of America. 101, 16, p. 5886-5891 6 p.

Research output: Contribution to journalArticle

226 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
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