dipeptidyl peptidase i

Summary

Summary: A cysteine-type peptidase that is CHLORINE dependent and functions maximally at acidic pHs. At neutral pH, it polymerizes esters, aryl- and dipeptide amides. EC 3.4.14.1.

Top Publications

  1. pmc Dipeptidyl peptidase I activates neutrophil-derived serine proteases and regulates the development of acute experimental arthritis
    April M Adkison
    Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri, USA
    J Clin Invest 109:363-71. 2002
  2. pmc The crystal structure of human dipeptidyl peptidase I (cathepsin C) in complex with the inhibitor Gly-Phe-CHN2
    Anne Mølgaard
    Centre for Crystallographic Studies, University of Copenhagen, Universitetsparken 5, DK 2100 Copenhagen Ø, Denmark
    Biochem J 401:645-50. 2007
  3. ncbi Dipeptidyl peptidase I: importance of progranzyme activation sequences, other dipeptide sequences, and the N-terminal amino group of synthetic substrates for enzyme activity
    Tinh V Tran
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA 30332 0400, USA
    Arch Biochem Biophys 403:160-70. 2002
  4. pmc Structure of human dipeptidyl peptidase I (cathepsin C): exclusion domain added to an endopeptidase framework creates the machine for activation of granular serine proteases
    D Turk
    Department of Biochemistry and Molecular Biology, Jozef Stefan Institute, Jamova 39, 1000 Ljubljana, Slovenia
    EMBO J 20:6570-82. 2001
  5. ncbi Papillon-Lefèvre syndrome: correlating the molecular, cellular, and clinical consequences of cathepsin C/dipeptidyl peptidase I deficiency in humans
    Christine T N Pham
    Department of Medicine, Washington University School of Medicine, Saint Louis, MO 63110, USA
    J Immunol 173:7277-81. 2004
  6. pmc Identification of cathepsin C mutations in ethnically diverse papillon-Lefèvre syndrome patients
    P S Hart
    University of Pittsburgh, Department of Human Genetics, Pittsburgh, PA 15261, USA
    J Med Genet 37:927-32. 2000
  7. ncbi Biochemical and mutational analyses of the cathepsin c gene (CTSC) in three North American families with Papillon Lefèvre syndrome
    Y Zhang
    Center for Craniofacial and Dental Genetics, University of Pittsburgh School of Dental Medicine, Pittsburgh, PA 15261, USA
    Hum Mutat 20:75. 2002
  8. pmc Residual active granzyme B in cathepsin C-null lymphocytes is sufficient for perforin-dependent target cell apoptosis
    Vivien R Sutton
    Cancer Immunology Program, Research Division, Peter MacCallum Cancer Centre, East Melbourne, Vic, Australia
    J Cell Biol 176:425-33. 2007
  9. ncbi Human recombinant pro-dipeptidyl peptidase I (cathepsin C) can be activated by cathepsins L and S but not by autocatalytic processing
    S W Dahl
    Prozymex A S, Dr Neergaards vej 17, DK 2970 Hørsholm, Denmark
    Biochemistry 40:1671-8. 2001
  10. ncbi Papillon-Lefèvre syndrome: mutations and polymorphisms in the cathepsin C gene
    A Nakano
    Department of Dermatology and Cutaneous Biology, Jefferson Medical College, Jefferson Institute of Molecular Medicine, 233 South 10th Street, Suite 450 BLSB, Philadelphia, PA 19107, USA
    J Invest Dermatol 116:339-43. 2001

Research Grants

  1. Inflammatory Proteases and Cardiac Repair after Myocardial Infarction
    Abdelkarim Sabri; Fiscal Year: 2013
  2. Mast Cells and the Host Response in the Lung
    Paul Wolters; Fiscal Year: 2007
  3. LLME TREATED T LYMPHOCYTE THERAPY AFTER ALLOGENEIC BMT
    Neal Flomenberg; Fiscal Year: 2002
  4. CYTOTOXIC CELLS AND ALLOIMMUNE RESPONSES
    Dwain Thiele; Fiscal Year: 2002
  5. MAST CELL CYSTEINE PROTEASES IN LUNG INFLAMMATION
    Paul Wolters; Fiscal Year: 2003
  6. PULMONARY EMPHYSEMA--ROLE OF SERINE PROTEINASES
    John Hoidal; Fiscal Year: 2000
  7. DPPI AND CELL MEDIATED IMMUNITY
    Christine Pham; Fiscal Year: 2001
  8. CYTOTOXIC CELLS AND ALLOIMMUNE RESPONSES
    Dwain Thiele; Fiscal Year: 2004
  9. Role of DPPI & Serine Proteases in Inflammatory Diseases
    Christine Pham; Fiscal Year: 2007

Scientific Experts

Detail Information

Publications132 found, 100 shown here

  1. pmc Dipeptidyl peptidase I activates neutrophil-derived serine proteases and regulates the development of acute experimental arthritis
    April M Adkison
    Department of Internal Medicine, Washington University School of Medicine, St Louis, Missouri, USA
    J Clin Invest 109:363-71. 2002
    ..To investigate the role of these proteases in inflammation, we generated a mouse deficient in dipeptidyl peptidase I (DPPI) and established that DPPI is required for the full activation of CG, NE, and PR3...
  2. pmc The crystal structure of human dipeptidyl peptidase I (cathepsin C) in complex with the inhibitor Gly-Phe-CHN2
    Anne Mølgaard
    Centre for Crystallographic Studies, University of Copenhagen, Universitetsparken 5, DK 2100 Copenhagen Ø, Denmark
    Biochem J 401:645-50. 2007
    hDDPI (human dipeptidyl peptidase I) is a lysosomal cysteine protease involved in zymogen activation of granule-associated proteases, including granzymes A and B from cytotoxic T-lymphocytes and natural killer cells, cathepsin G and ..
  3. ncbi Dipeptidyl peptidase I: importance of progranzyme activation sequences, other dipeptide sequences, and the N-terminal amino group of synthetic substrates for enzyme activity
    Tinh V Tran
    School of Chemistry and Biochemistry, Georgia Institute of Technology, Atlanta, GA 30332 0400, USA
    Arch Biochem Biophys 403:160-70. 2002
    The broadly reactive cysteine protease dipeptidyl peptidase I (DPPI, cathepsin C) is thought to activate all progranzymes (zymogens of lymphocyte serine proteases) to form mature granzymes...
  4. pmc Structure of human dipeptidyl peptidase I (cathepsin C): exclusion domain added to an endopeptidase framework creates the machine for activation of granular serine proteases
    D Turk
    Department of Biochemistry and Molecular Biology, Jozef Stefan Institute, Jamova 39, 1000 Ljubljana, Slovenia
    EMBO J 20:6570-82. 2001
    b>Dipeptidyl peptidase I (DPPI) or cathepsin C is the physiological activator of groups of serine proteases from immune and inflammatory cells vital for defense of an organism...
  5. ncbi Papillon-Lefèvre syndrome: correlating the molecular, cellular, and clinical consequences of cathepsin C/dipeptidyl peptidase I deficiency in humans
    Christine T N Pham
    Department of Medicine, Washington University School of Medicine, Saint Louis, MO 63110, USA
    J Immunol 173:7277-81. 2004
    ..proteases depends on the N-terminal processing activity of the lysosomal cysteine protease cathepsin C/dipeptidyl peptidase I (DPPI)...
  6. pmc Identification of cathepsin C mutations in ethnically diverse papillon-Lefèvre syndrome patients
    P S Hart
    University of Pittsburgh, Department of Human Genetics, Pittsburgh, PA 15261, USA
    J Med Genet 37:927-32. 2000
    ..To date, 13 different cathepsin C mutations have been reported in PLS patients, all of which are homozygous for a given mutation, reflecting consanguinity...
  7. ncbi Biochemical and mutational analyses of the cathepsin c gene (CTSC) in three North American families with Papillon Lefèvre syndrome
    Y Zhang
    Center for Craniofacial and Dental Genetics, University of Pittsburgh School of Dental Medicine, Pittsburgh, PA 15261, USA
    Hum Mutat 20:75. 2002
    ..These mutations altered restriction enzyme sites in the highly conserved CTSC gene. Sequence analysis of CTSC exon 3 confirmed the previously reported p.T153I polymorphism in 4 of the 5 ethnically diverse populations studied...
  8. pmc Residual active granzyme B in cathepsin C-null lymphocytes is sufficient for perforin-dependent target cell apoptosis
    Vivien R Sutton
    Cancer Immunology Program, Research Division, Peter MacCallum Cancer Centre, East Melbourne, Vic, Australia
    J Cell Biol 176:425-33. 2007
    ..Cathepsin C deficiency also results in considerably milder immune deficiency than perforin or granzyme A/B deficiency...
  9. ncbi Human recombinant pro-dipeptidyl peptidase I (cathepsin C) can be activated by cathepsins L and S but not by autocatalytic processing
    S W Dahl
    Prozymex A S, Dr Neergaards vej 17, DK 2970 Hørsholm, Denmark
    Biochemistry 40:1671-8. 2001
    Human dipeptidyl peptidase I was expressed in the insect cell/baculovirus system and purified in its active (rhDPPI) and precursor (pro-rhDPPI) forms...
  10. ncbi Papillon-Lefèvre syndrome: mutations and polymorphisms in the cathepsin C gene
    A Nakano
    Department of Dermatology and Cutaneous Biology, Jefferson Medical College, Jefferson Institute of Molecular Medicine, 233 South 10th Street, Suite 450 BLSB, Philadelphia, PA 19107, USA
    J Invest Dermatol 116:339-43. 2001
    ..Addition of these cathepsin C gene mutations into the expanding Papillon-Lefèvre syndrome mutation database allows further development of genotype/phenotype correlations towards understanding this severe genodermatosis...
  11. ncbi A novel mutation of the cathepsin C gene in Papillon-Lefèvre syndrome
    Vanessa F Cury
    Department of Oral Surgery and Pathology, School of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil
    J Periodontol 73:307-12. 2002
    ..However, genotypic and phenotypic correlation has not been established. In the present study we investigated the CTSC gene in a Brazilian cohort affected by PLS...
  12. ncbi Novel point mutations, deletions, and polymorphisms in the cathepsin C gene in nine families from Europe and North Africa with Papillon-Lefèvre syndrome
    C Lefevre
    Centre National de Genotypage, Evry, France
    J Invest Dermatol 117:1657-61. 2001
    ..Hum Mutat 17:152-153, 2001)...
  13. ncbi Cloning and characterization of the cDNA encoding mouse dipeptidyl peptidase I (cathepsin C)
    M J McGuire
    Department of Internal Medicine, The University of Texas Southwestern Medical Center, Dallas 75235 8577, USA
    Biochim Biophys Acta 1351:267-73. 1997
    The mouse dipeptidyl peptidase I cDNA has been cloned and sequenced and patterns of DPPI mRNA expression in various tissues characterized...
  14. pmc Dipeptidyl peptidase I is required for the processing and activation of granzymes A and B in vivo
    C T Pham
    Division of Rheumatology, Department of Internal Medicine, Washington University School of Medicine, St Louis, MO 63110 1093, USA
    Proc Natl Acad Sci U S A 96:8627-32. 1999
    b>Dipeptidyl peptidase I (DPPI) is a lysosomal cysteine protease that has been implicated in the processing of granzymes, which are neutral serine proteases exclusively expressed in the granules of activated cytotoxic lymphocytes...
  15. ncbi Loss-of-function mutations in the cathepsin C gene result in periodontal disease and palmoplantar keratosis
    C Toomes
    Department of Medical Genetics, St Mary s Hospital, Manchester, UK
    Nat Genet 23:421-4. 1999
    ..In two of these families we used a functional assay to demonstrate an almost total loss of cathepsin C activity in PLS patients and reduced activity in obligate carriers...
  16. pmc Dipeptidyl peptidase I-dependent neutrophil recruitment modulates the inflammatory response to Sendai virus infection
    Antonina M Akk
    Division of Rheumatology, Department of Internal Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Immunol 180:3535-42. 2008
    ..In this study, we show that the absence of dipeptidyl peptidase I (DPPI), a lysosomal cysteine protease found in neutrophils, dampens the acute inflammatory response and ..
  17. ncbi Cathepsin C gene: First compound heterozygous patient with Papillon-Lefèvre syndrome and a novel symptomless mutation
    L M Allende
    Department of Immunology, Hospital 12 de Octubre, Universidad Complutense, Madrid, Spain
    Hum Mutat 17:152-3. 2001
    ..On the other hand, PLS patients are ameliorated by retinoids, which indicates that retinoids may be used as therapeutic agents in this immune system deficiency...
  18. pmc Mutations of the cathepsin C gene are responsible for Papillon-Lefèvre syndrome
    T C Hart
    Department of Pediatrics, Section of Medical Genetics, Wake Forest University School of Medicine, Winston Salem, North Carolina, USA
    J Med Genet 36:881-7. 1999
    ..A more complete understanding of the functional physiology of cathepsin C carries significant implications for understanding normal and abnormal skin development and periodontal disease susceptibility...
  19. ncbi Human dipeptidyl-peptidase I. Gene characterization, localization, and expression
    N V Rao
    Department of Internal Medicine, Division of Respiratory, Critical Care, and Occupational Medicine, University of Utah Health Sciences Center and Veterans Administration Medical Center, Salt Lake City, Utah 84132, USA
    J Biol Chem 272:10260-5. 1997
    ..The results provide initial insights into the molecular basis for the regulation of human dipeptidyl-peptidase I...
  20. ncbi Dipeptidyl peptidase I regulates the development of collagen-induced arthritis
    Ying Hu
    Washington University School of Medicine, St Louis, MO 63110, USA
    Arthritis Rheum 52:2553-8. 2005
    To examine the role of dipeptidyl peptidase I (DPPI), a widely expressed lysosomal cysteine protease, in the development of collagen-induced arthritis (CIA) in mice.
  21. ncbi A family with Papillon-Lefevre syndrome reveals a requirement for cathepsin C in granzyme B activation and NK cell cytolytic activity
    Josephine L Meade
    Leeds Institute of Molecular Medicine, University of Leeds, St James s University Hospital, Leeds LS9 7TF, United Kingdom
    Blood 107:3665-8. 2006
    ..This is the first documented example of a human mutation affecting granzyme B activity and highlights the importance of cathepsin C in human NK cell function...
  22. pmc Protease-activated receptor 2, dipeptidyl peptidase I, and proteases mediate Clostridium difficile toxin A enteritis
    Graeme S Cottrell
    Center for the Neurobiology of Digestive Disease, Department of Surgery, University of California, San Francisco, San Francisco, California 94143 0660, USA
    Gastroenterology 132:2422-37. 2007
    ..We studied the role of protease-activated receptor 2 (PAR(2)) and its activating enzymes, trypsins and tryptase, in Clostridium difficile toxin A (TxA)-induced enteritis...
  23. pmc Critical role of dipeptidyl peptidase I in neutrophil recruitment during the development of experimental abdominal aortic aneurysms
    Monica B Pagano
    Department of Surgery, Section of Vascular Surgery, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63124, USA
    Proc Natl Acad Sci U S A 104:2855-60. 2007
    b>Dipeptidyl peptidase I (DPPI) is a lysosomal cysteine protease critical for the activation of granule-associated serine proteases, including neutrophil elastase, cathepsin G, and proteinase 3...
  24. pmc Haim-Munk syndrome and Papillon-Lefèvre syndrome are allelic mutations in cathepsin C
    T C Hart
    University of Pittsburgh, School of Dental Medicine, Department of Oral Medicine Pathology, 628 Salk Hall, 3501 Terrace Street, Pittsburgh, PA 15261, USA
    J Med Genet 37:88-94. 2000
    ..We also report a mutation of the same exon 6 CTSC codon (2126C-->T) in a Turkish family with classical PLS. These findings provide evidence that PLS and HMS are allelic variants of cathepsin C gene mutations...
  25. ncbi Purification and characterization of dipeptidyl peptidase I from human spleen
    M J McGuire
    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas 75235
    Arch Biochem Biophys 295:280-8. 1992
    The lysosomal hydrolase, dipeptidyl peptidase I (DPPI), was purified from human spleen and its enzymatic activity characterized...
  26. ncbi A selective activity-based probe for the papain family cysteine protease dipeptidyl peptidase I/cathepsin C
    Fang Yuan
    Department of Pathology, and Microbiology and Immunology, Stanford School of Medicine, 300 Pasteur Drive, Stanford, California 94305, USA
    J Am Chem Soc 128:5616-7. 2006
    b>Dipeptidyl peptidase I is involved in the activation of a number of disease-related proteases by removal of N-terminal prodipeptides...
  27. pmc Localisation of a gene for prepubertal periodontitis to chromosome 11q14 and identification of a cathepsin C gene mutation
    T C Hart
    Department of Oral Medicine Pathology, University of Pittsburgh, School of Dental Medicine, 614 Salk Hall, 3501 Terrace Street, Pittsburgh, PA 15261, USA
    J Med Genet 37:95-101. 2000
    ..This is the first reported gene mutation for non-syndromic periodontitis and shows that non-syndromic PPP is an allelic variant of the type IV palmoplantar ectodermal dysplasias...
  28. pmc Free-thiol Cys331 exposed during activation process is critical for native tetramer structure of cathepsin C (dipeptidyl peptidase I)
    Martin Horn
    Institute of Organic Chemistry and Biochemistry, Czech Academy of Sciences, 16610 Praha, Czech Republic
    Protein Sci 11:933-43. 2002
    ..In conclusion, the mechanism of tetramer formation as well as specific posttranslational processing segregates CC in the family of papain proteases...
  29. pmc Mast cell dipeptidyl peptidase I mediates survival from sepsis
    Jon Mallen-St Clair
    Department of Medicine and the Cardiovascular Research Institute, University of California, San Francisco, California 94143 0911, USA
    J Clin Invest 113:628-34. 2004
    Sepsis is a common, life-threatening disease for which there is little treatment. The cysteine protease dipeptidyl peptidase I (DPPI) activates granule-associated serine proteases, several of which play important roles in host responses ..
  30. ncbi Identification of a novel cathepsin C mutation (p.W185X) in a Brazilian kindred with Papillon-Lefèvre syndrome
    P S Hart
    Department of Human Genetics, Graduate School of Public Health, University of Pittsburgh, 572A Scaife Hall, 3550 Terrace St, PA 15261, USA
    Mol Genet Metab 76:145-7. 2002
    ..This family segregates a novel PLS-related mutation, p.W185X, that is associated with a complete loss of enzymatic activity...
  31. ncbi Selective inhibition of dipeptidyl peptidase I, not caspases, prevents the partial processing of procaspase-3 in CD3-activated human CD8(+) T lymphocytes
    Nicolas Bidere
    Laboratoire de Greffes d Epithéliums et Régulation de l Activation Lymphocytaire, Unité INSERM 542, Hopital Paul Brousse, 94807 Villejuif, France
    J Biol Chem 277:32339-47. 2002
    ..dmk, a selective inhibitor of dipeptidyl peptidase I, was effective...
  32. ncbi Inhibition of dipeptidyl peptidase I in the human mast cell line HMC-1: blocked activation of tryptase, but not of the predominant chymotryptic activity
    Parimal D Sheth
    Immunopharmacology Group, Division of Infection, Immunity and Repair, University of Southampton Medical School, Southampton General Hospital, Southampton SO16 6YD, UK
    Biochem Pharmacol 66:2251-62. 2003
    ..The cysteinyl protease dipeptidyl peptidase I (DPPI, cathepsin C) can activate the corresponding proenzymes in cell-free systems, but it is unknown ..
  33. ncbi Molecular cloning, chromosomal localization, and expression of murine dipeptidyl peptidase I
    C T Pham
    Department of Internal Medicine, Washington University Medical School, St Louis, Missouri 63110 1093, USA
    J Biol Chem 272:10695-703. 1997
    b>Dipeptidyl peptidase I (DPPI) is a lysosomal cysteine protease that catalyzes the sequential removal of dipeptides from the amino termini of various protein substrates...
  34. ncbi Dipeptidyl peptidase I is essential for activation of mast cell chymases, but not tryptases, in mice
    P J Wolters
    Department of Medicine and Cardiovascular Research Institute, University of California, San Francisco, California 94143 0911, USA
    J Biol Chem 276:18551-6. 2001
    b>Dipeptidyl peptidase I (DPPI) is the sole activator in vivo of several granule-associated serine proteases of cytotoxic lymphocytes. In vitro, DPPI also activates mast cell chymases and tryptases...
  35. pmc Cathepsins L and S are not required for activation of dipeptidyl peptidase I (cathepsin C) in mice
    Jon Mallen-St Clair
    Department of Medicine and Cardiovascular Research Institute, University of California, San Francisco, CA 94143 0111, USA
    Biol Chem 387:1143-6. 2006
    The cysteine protease dipeptidyl peptidase I (DPPI) activates granule-associated immune-cell serine proteases...
  36. ncbi Design and evaluation of inhibitors for dipeptidyl peptidase I (Cathepsin C)
    Chih Min Kam
    School of Chemistry and Biochemistry and the Petit Institute for Bioscience and Bioengineering, Georgia Institute of Technology, Atlanta, GA 30332 0400, USA
    Arch Biochem Biophys 427:123-34. 2004
    b>Dipeptidyl peptidase I (DPPI, cathepsin C) is a lysosomal cysteine protease that can activate zymogens of several different serine proteases by one step or sequential removal of dipeptides from the N-termini of the pro-protease protein ..
  37. ncbi Processing of procarboxypeptidase A and other zymogens in murine mast cells
    M M Dikov
    Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee 37232 0111
    J Biol Chem 269:25897-904. 1994
    ..Thus, mast cell carboxypeptidase, tryptase, and chymase zymogens are each processed to their active forms by different mechanisms...
  38. ncbi Regulated expression, processing, and secretion of dog mast cell dipeptidyl peptidase I
    P J Wolters
    Department of Medicine and the Cardiovascular Research Institute, University of California, San Francisco, California 94143 0911, USA
    J Biol Chem 273:15514-20. 1998
    b>Dipeptidyl peptidase I (DPPI) is a cysteine protease found predominantly in myelomonocytic cells, cytotoxic T-cells, and mast cells...
  39. ncbi Demonstration of altered splicing with the IVS3-1G --> a mutation of cathepsin C
    Mohamad Nusier
    Department of Biochemistry and Molecular Biology, Jordan University of Science and Technology, Irbid, Jordan
    Mol Genet Metab 75:280-3. 2002
    ..Sequencing of the mutant transcript revealed that it lacked exon 3, resulting in a frameshift and introduction of a premature termination codon...
  40. pmc Evidence of a founder effect for four cathepsin C gene mutations in Papillon-Lefèvre syndrome patients
    Y Zhang
    Department of Oral Biology, University of Pittsburgh School of Dental Medicine, 614 Salk Hall, 3501 Terrace Street, Pittsburgh, PA 15261, USA
    J Med Genet 38:96-101. 2001
    ..Identification of these six short tandem repeat polymorphisms that span the cathepsin C gene will permit haplotype analyses to determine other founder haplotypes of cathepsin C mutations in additional PLS families...
  41. ncbi New developments in the genetics and activation of mast cell proteases
    George H Caughey
    Department of Medicine, Cardiovascular Research Institute, University of California at San Francisco, San Francisco, CA 94143 0911, USA
    Mol Immunol 38:1353-7. 2002
    ..New biological tools, including the dipeptidyl peptidase I (DPPI)-null mouse in which the entire class of mast cell chymases appears to be functionally knocked out, ..
  42. ncbi Papillon Lefevre syndrome
    S V Dhadke
    Department of Medicine Dr VM Medical College Solapur
    J Assoc Physicians India 54:246-7. 2006
    ..Pyogenic liver abscess is an increasingly recognized complication. We report a new case of this association and review the current literature...
  43. ncbi Novel mutations in the cathepsin C gene in patients with pre-pubertal aggressive periodontitis and Papillon-Lefèvre syndrome
    B Noack
    J Dent Res 83:368-70. 2004
    ..947T>G, c.1268G>C) in a PLS patient. We conclude that at least a subset of pre-pubertal AP is due to CTSC mutations and therefore may be an allelic variant of PLS...
  44. ncbi Phenotypic variation and allelic heterogeneity in young patients with Papillon-Lefèvre syndrome
    Christer Ullbro
    Department of Dentistry, King Faisal Specialist Hospital, Research Centre, Riyadh, Saudi Arabia
    Acta Derm Venereol 86:3-7. 2006
    ..Young girls displayed significantly less palmoplantar hyperkeratosis ( p < 0.05) than young boys. In conclusion, considerable phenotypic heterogeneity was observed within the two cardinal mutations and in the 189/189 genotype...
  45. ncbi Mast cell cathepsins C and S control levels of carboxypeptidase A and the chymase, mouse mast cell protease 5
    Frida Henningsson
    Swedish University of Agricultural Sciences, Department of Veterinary Medical Chemistry, The Biomedical Center, Box 575, S 751 23 Uppsala, Sweden
    Biol Chem 384:1527-31. 2003
    ..The present study thus indicates novel roles for cathepsins C and S in regulating the levels of stored proteases in the mast cell secretory granules...
  46. ncbi Late-onset Papillon-Lefèvre syndrome without alteration of the cathepsin C gene
    Ulrike Pilger
    Department of Dermatology, Karl Franzens University, Graz, Austria
    J Am Acad Dermatol 49:S240-3. 2003
    ..Our patient with late-onset variation of PLS, however, did not show a mutation in the cathepsin C gene. Thus, we suspect that there is another genetic cause for the late-onset forms of PLS...
  47. ncbi New syndrome of hypotrichosis, striate palmoplantar keratoderma, acro-osteolysis and periodontitis not due to mutations in cathepsin C
    M A M van Steensel
    Department of Dermatology, University Medical Centre St Radboud Nijmegen, PO Box 9101, 6500 HB Nijmegen, The Netherlands
    Br J Dermatol 147:575-81. 2002
    ..We propose that our patients suffer from a new syndrome possibly caused by mutations in a gene that has a functional or structural relation with CTSC...
  48. ncbi Purification and characterization of cathepsin J from rat liver
    T Nikawa
    Division of Enzyme Chemistry, University of Tokushima, Japan
    Eur J Biochem 204:381-93. 1992
    ..J. & Ellis, S. (1964) Biochem. Biophys. Res. Commun. 16, 135-140]. Furthermore, antiserum against rat liver cathepsin C reacted with rat liver cathepsin J. These findings suggested that cathepsin J is identical with cathepsin C...
  49. pmc Carbohydrate- and conformation-dependent cargo capture for ER-exit
    Christian Appenzeller-Herzog
    Department of Pharmacology and Neurobiology, Biozentrum, University of Basel, CH 4056 Basel, Switzerland
    Mol Biol Cell 16:1258-67. 2005
    ..To our knowledge this is the first documentation of an ER-exit signal in soluble cargo in conjunction with its decoding by a transport receptor...
  50. ncbi Cathepsin C involvement in the aetiology of Papillon-Lefevre syndrome
    Cristina Frezzini
    Int J Paediatr Dent 14:466-7. 2004
  51. ncbi A novel mutation of the cathepsin C gene in a thai family with Papillon-Lefevre syndrome
    Hiroshi Nitta
    Department of Comprehensive Oral Health Care, Behavioral Dentistry, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan
    J Periodontol 76:492-6. 2005
    ..This study demonstrates the clinical manifestations and CTSC mutational and enzymatic activity analyses in a 5-year-old Thai male PLS patient and his parents...
  52. ncbi Papillon-Lefèvre syndrome and malignant melanoma. A high incidence of melanoma development in Japanese palmoplantar keratoderma patients
    Koji Nakajima
    Department of Dermatology, Hirosaki University School of Medicine, Hirosaki, Japan
    Dermatology 217:58-62. 2008
    ..This tendency might be attributable to the high frequency of acral lentiginous melanoma in Japanese subjects, in contrast to a lower frequency of this subtype in Caucasians...
  53. ncbi Gene symbol: CTSC. Disease: Papillon-Lefevre syndrome
    W Beertsen
    Department of Periodontology, Academic Centre for Dentistry Amsterdam, Universiteit van Amsterdam, The Netherlands
    Hum Genet 116:545. 2005
  54. ncbi Loss-of-function mutations in cathepsin C in two families with Papillon-Lefèvre syndrome are associated with deficiency of serine proteinases in PMNs
    Susanne F de Haar
    Department of Periodontology, Academic Centre for Dentistry Amsterdam ACTA, Universiteit van Amsterdam, Amsterdam, The Netherlands
    Hum Mutat 23:524. 2004
    ..Serine proteinases are supposed to be important in both the innate and adaptive immune systems. Their absence in PLS patients could explain the inadequate defense to periodontal infection...
  55. ncbi Genes involved in Alzheimer's disease, a survey of possible candidates
    Alessandro Serretti
    Department of Psychiatry, Vita Salute University School of Medicine, Fondazione Centro San Raffaele del Monte Tabor, Milan, Italy
    J Alzheimers Dis 7:331-53. 2005
    ..The aim of this review is to explore genetics of AD...
  56. ncbi Papillon-Lefèvre syndrome: case report and review of the literature
    Shahbaz A Janjua
    Ayza Skin and Research Center, Lalamusa, Pakistan
    Dermatol Online J 10:13. 2004
    ..These findings are consistent with Papillon-Lefèvre syndrome. The clinical presentation, differential diagnosis, complications and management of this syndrome are discussed...
  57. ncbi Novel mutations of cathepsin C gene in two Chinese patients with Papillon-Lefèvre syndrome
    Y Yang
    Department of Pedodontics, Peking University, Beijing, People s Republic of China
    J Dent Res 86:735-8. 2007
    ..851G>A and c.112delCCTG) in the other patient. Our novel discovery indicates that the phenotypes observed in these two patients are due to the CTSC gene mutation...
  58. ncbi Clinical, genetic, and biochemical findings in two siblings with Papillon-Lefèvre Syndrome
    N Arzu Cagli
    Department of Periodontology, Faculty of Dentistry, Selcuk University, Konya, Turkey
    J Periodontol 76:2322-9. 2005
    ..Cathepsin C (CTSC) gene mutations are etiologic for PLS. The resultant loss of CTSC function is responsible for the severe periodontal destruction seen clinically...
  59. ncbi Functional Cathepsin C mutations cause different Papillon-Lefèvre syndrome phenotypes
    Barbara Noack
    Department of Conservative Dentistry, University Hospital Carl Gustav Carus, TU Dresden, Germany
    J Clin Periodontol 35:311-6. 2008
    ..This study aimed to identify CTSC mutations in different PLS phenotypes, including atypical forms and isolated pre-pubertal aggressive periodontitis (PAP)...
  60. ncbi The role of cathepsin C in Papillon-Lefèvre syndrome, prepubertal periodontitis, and aggressive periodontitis
    Chelsee Hewitt
    Department of Medical Genetics University of Manchester, Manchester, UK
    Hum Mutat 23:222-8. 2004
    ..These data suggest that prepubertal periodontitis is a genetically heterogeneous disease that, in some families, just represents a partially penetrant PLS...
  61. ncbi Cargo selectivity of the ERGIC-53/MCFD2 transport receptor complex
    Beat Nyfeler
    Biozentrum, University of Basel, CH 4056 Basel, Switzerland
    Traffic 7:1473-81. 2006
    ..The results indicate that ERGIC-53 can bind cargo glycoproteins in an MCFD2-independent fashion and suggest that MCFD2 is a recruitment factor for blood coagulation factors V and VIII...
  62. ncbi Cathepsin Cs are key for the intracellular survival of the protozoan parasite, Toxoplasma gondii
    Xuchu Que
    Department of Pathology, University of California, San Diego, California 92103 8416, USA
    J Biol Chem 282:4994-5003. 2007
    ..gondii in the chick embryo model of toxoplasmosis. Thus, cathepsin Cs are critical to T. gondii growth and differentiation, and their unique specificities could be exploited to develop novel chemotherapeutic agents...
  63. ncbi Use of compact, porous units with immobilized ligands with high molecular masses in affinity chromatography and enzymatic conversion of substrates with high and low molecular masses
    D Josic
    Octapharma Pharmazeutika Produktionsges, mbH, Wien, Austria
    J Chromatogr A 803:61-71. 1998
    ..The enzyme dipeptidyl peptidase i.v...
  64. ncbi Cysteine peptidases of mammals: their biological roles and potential effects in the oral cavity and other tissues in health and disease
    D P Dickinson
    Medical College of Georgia, School of Dentistry, Department of Oral Biology, and Maxillofacial Pathology, Augusta 30912, USA
    Crit Rev Oral Biol Med 13:238-75. 2002
    ..Human cathepsins are best known from the ubiquitously expressed lysosomal cathepsins B, H, and L, and dipeptidyl peptidase I (DPP I), which until recently were considered to mediate primarily "housekeeping" functions in the cell...
  65. pmc Lysosomal degradation of cholecystokinin-(29-33)-amide in mouse brain is dependent on tripeptidyl peptidase-I: implications for the degradation and storage of peptides in classical late-infantile neuronal ceroid lipofuscinosis
    Francesca Bernardini
    Department of Cellular Pathology, St George s Hospital Medical School, Cranmer Terrace, London SW17 0RE, UK
    Biochem J 366:521-9. 2002
    ..Other tissues are spared because they express another peptidase, DPP-I, which has extensive activity on peptides and can compensate for the loss of TPP-I...
  66. ncbi Functional expression and characterization of Schistosoma mansoni cathepsin B and its trans-activation by an endogenous asparaginyl endopeptidase
    Mohammed Sajid
    Department of Pathology, Tropical Disease Research Unit and Sandler Centre for Basic Research in Parasitic Diseases, University of California San Francisco, Box 0511, San Francisco, CA 94143, USA
    Mol Biochem Parasitol 131:65-75. 2003
    ..This study characterizes the major digestive cysteine peptidase in schistosomes and defines novel trans-processing events required to activate the SmCB1 zymogen in vitro which may facilitate the digestive process in vivo...
  67. ncbi Removal of N-terminal polyhistidine tags from recombinant proteins using engineered aminopeptidases
    J Pedersen
    UNIZYME Laboratories, Dr Neergaards vej 17, Horsholm, DK 2970, Denmark
    Protein Expr Purif 15:389-400. 1999
    ..The method is based on the use of the aminopeptidase dipeptidyl peptidase I (DPPI), either alone or in combination with glutamine cyclotransferase (GCT) and pyroglutamyl ..
  68. ncbi Lysosomal enzyme activities: new potential markers for Sjögren's syndrome
    Nicolette Sohar
    Department of Ophthalmology, University of Szeged, A Szent Györgyi Medical and Pharmaceutical Center, Szeged, Hungary
    Clin Biochem 38:1120-6. 2005
    ..To evaluate the changes in lysosomal enzyme activities in leukocytes of patients with Sjögren's syndrome...
  69. ncbi Therapeutic utility and medicinal chemistry of cathepsin C inhibitors
    Daniel Guay
    Department of Medicinal Chemistry, Merck Frosst Centre for Therapeutic Research, Kirkland, Quebec, Canada
    Curr Top Med Chem 10:708-16. 2010
    The lysosomal cysteine protease cathepsin C (Cat C), also known as dipeptidyl peptidase I, activates a number of granule-associated serine proteases with pro-inflammatory and immune functions by removal of their inhibitory N-terminal ..
  70. pmc Neutrophil elastase, proteinase 3, and cathepsin G as therapeutic targets in human diseases
    Brice Korkmaz
    INSERM U 618 Protéases et Vectorisation Pulmonaires, Universite Francois Rabelais, Faculte de Medecine, 10 boulevard Tonnelle, Tours, France
    Pharmacol Rev 62:726-59. 2010
    ..All three proteases are affected by mutations of the gene (CTSC) encoding dipeptidyl peptidase I, a protease required for activation of their proform before storage in cytoplasmic granules...
  71. pmc Increased collagenase and dipeptidyl peptidase I activity in leucocytes from healthy elderly people
    L Llorente
    Department of Immunology and Rheumatology, Instituto Nacional dela Nutrición Salvador Zubirán, Mexico City, Mexico
    Clin Exp Immunol 116:425-9. 1999
    ..substrates employed were for oxidative burst, L-aminopeptidase, collagenase, cathepsin B, C, D and, G and dipeptidyl peptidase I. The intracellular enzyme activity assessed by flow cytometry in leucocytes from young and elderly women ..
  72. ncbi Variation of proteolytic activity in ewe during in vivo digestion of two pea-based diets differing by their fermentability characteristics
    M Sales-Duval
    ENSAIA, Laboratoire de Sciences Animales, Vandruvre les Nancy
    Reprod Nutr Dev 41:297-308. 2001
    ..diet II, exopeptidase activities increased more in the liquid phase, especially leucine aminopeptidase and Dipeptidyl peptidase I (DPP-I), and the diversity of endopeptidase activities increased in the particulate phase...
  73. ncbi Cell-type specificity of l-leucyl l-leucine methyl ester
    Y Kobayashi
    Department of Biomolecular Science, Faculty of Science, Toho University, Chiba, Japan
    Biochem Biophys Res Commun 272:687-90. 2000
    ..methyl ester (LeuLeuOMe) is a lysosomotropic agent which is converted to a membranolytic compound by dipeptidyl peptidase I and kills human leukocytes such as CD8+ T cells and monocytes but not B cells...
  74. ncbi Inhibitors of cathepsin C (dipeptidyl peptidase I)
    Dramane I Laine
    GlaxoSmithKline, Respiratory CEDD, King of Prussia, PA 19406, USA
    Expert Opin Ther Pat 20:497-506. 2010
    Cathepsin C (dipeptidyl peptidase I) plays a key role in the activation of several degradative enzymes linked to tissue destruction in inflammatory diseases...
  75. ncbi Innate immune response mechanisms in non-insulin dependent diabetes mellitus patients assessed by flow cytoenzymology
    L Llorente
    Department of Immunology and Rheumatology, Instituto Nacional de la Nutricion Salvador Zubiran, Vasco de Quiroga 15, Tlalpan CP 14000, Mexico, D F, Mexico
    Immunol Lett 74:239-44. 2000
    ..Other mechanisms - either from the non-specific or specific immune response - deserve investigation to establish if diabetic patients are more susceptible to infectious diseases...
  76. ncbi Expression and characterization of recombinant mast cell tryptase
    H Chan
    Department of Molecular Biology, Department of Enzymology, Department of Pharmacology, Axys Pharmaceuticals, Inc, 180 Kimball Way, South San Francisco, California, 94080, USA
    Protein Expr Purif 15:251-7. 1999
    ..of active recombinant tryptase required a two column purification protocol and included a heparin- and dipeptidyl peptidase I-dependent activation step...
  77. pmc Fas and TNFR1, but not cytolytic granule-dependent mechanisms, mediate clearance of murine liver adenoviral infection
    Marwan S Abougergi
    Department of Internal Medicine, The University of Texas Southwestern Medical Center, Dallas, TX 75390 9151, USA
    Hepatology 41:97-105. 2005
    ..of secreted or intracellular adenoviral gene products was not delayed in perforin-deficient mice or dipeptidyl peptidase I-deficient mice, which fail to process and activate granzyme A or granzyme B...
  78. ncbi Bifidobacterium bifidum monoassociation of gnotobiotic mice: effect on enterocyte brush-border enzymes
    H Kozakova
    Department of Immunology and Gnotobiology, Institute of Microbiology, Academy of Sciences of the Czech Republic, 549 22 Novy Hradek, Czechia
    Folia Microbiol (Praha) 46:573-6. 2001
    ..This effect of B. bifidum supplementation was less pronounced for alkaline phosphatase, sucrase, glucoamylase and dipeptidyl peptidase i.v. in immature gut of weaned mice than of 2-month-old ones.
  79. ncbi Novel semicarbazide-derived inhibitors of human dipeptidyl peptidase I (hDPPI)
    Jon Bondebjerg
    Combio A S, Vesterbrogade 188, DK 1800 Frederiksberg C, Denmark
    Bioorg Med Chem 13:4408-24. 2005
    Human dipeptidyl peptidase I (hDPPI, cathepsin C, EC 3.4.14.1) is a novel putative drug target for the treatment of inflammatory diseases...
  80. ncbi Identification of genetic risk factors for periodontitis and possible mechanisms of action
    Bruno G Loos
    Department of Periodontology, Acadcemic Center for Dentistry Amsterdam ACTA, University of Amsterdam, The Netherlands
    J Clin Periodontol 32:159-79. 2005
    ..To review the literature for genetic risk factors associated with periodontitis...
  81. ncbi Papillon-Lefévre syndrome: a highly-suspected case
    Atsushi Ikeshima
    Department of Radiology, Nihon University School of Dentistry at Matsudo, Chiba, Japan
    J Oral Sci 48:257-60. 2006
    ..However, the special precautions normally applied to prevent infections in PLS patients undergoing dental treatment were taken...
  82. pmc Role of NADPH oxidase versus neutrophil proteases in antimicrobial host defense
    R Robert Vethanayagam
    Department of Medicine, Roswell Park Cancer Institute, Buffalo, New York, United States of America
    PLoS ONE 6:e28149. 2011
    ..neutrophil elastase (NE)(-/-)×cathepsin G (CG)(-/-) mice and lysosomal cysteine protease cathepsin C/dipeptidyl peptidase I (DPPI)-deficient mice that are defective in neutrophil serine protease activation demonstrated no ..
  83. ncbi Neutrophil proteinase 3 and dipeptidyl peptidase I (cathepsin C) as pharmacological targets in granulomatosis with polyangiitis (Wegener granulomatosis)
    Brice Korkmaz
    Centre d Etude des Pathologies Respiratoires, INSERM U 1100 EA 6305, 37032 Tours, France
    Semin Immunopathol 35:411-21. 2013
    ..proteinase 3 (PR3), and neutrophil serine protease 4 are four hematopoietic serine proteases activated by dipeptidyl peptidase I during neutrophil maturation and are mainly stored in cytoplasmic azurophilic granules...
  84. pmc Cystatin F is a cathepsin C-directed protease inhibitor regulated by proteolysis
    Garth Hamilton
    Division of Cell Biology and Immunology, College of Life Sciences, University of Dundee, Dundee, UK
    EMBO J 27:499-508. 2008
    ..Thus, cystatin F is a latent protease inhibitor itself regulated by proteolysis in the endocytic pathway. By targeting cathepsin C, it may regulate diverse immune cell effector functions...
  85. ncbi Dermatologic, periodontal, and skeletal manifestations of Haim-Munk syndrome in two siblings
    Shahbaz A Janjua
    Ayza Skin and Research Center, Lalamusa, Pakistan
    J Am Acad Dermatol 58:339-44. 2008
    ....
  86. ncbi Carica papaya glutamine cyclotransferase belongs to a novel plant enzyme subfamily: cloning and characterization of the recombinant enzyme
    S W Dahl
    Unizyme Laboratories A S, Dr Neergaards vej 17, Horsholm, Denmark
    Protein Expr Purif 20:27-36. 2000
    ..was correctly processed by the host signal peptidase and the His-tag could subsequently be removed with dipeptidyl peptidase I. The expressed products were characterized by activity assays, SDS-PAGE, N-terminal amino acid sequencing, ..
  87. ncbi Effect of epsilon-aminocaproylaminoacids on the activity of proteolytic enzymes
    I Bruzgo
    Institute of Chemistry, , Poland
    Acta Pol Pharm 58:137-40. 2001
    ..Slight inhibition of trypsin and chymotrypsin activity was observed only at high concentrations of these compounds. All tested dipeptides did not influence activities of cathepsin B, cathepsin C and cathepsin D...
  88. ncbi The human gene FALL39 and processing of the cathelin precursor to the antibacterial peptide LL-37 in granulocytes
    G H Gudmundsson
    Department of Microbiology, Stockholm University, Sweden
    Eur J Biochem 238:325-32. 1996
    ..To obtain LL-37 for antibacterial assays, synthetic FA-LL-37 was degraded with dipeptidyl-peptidase I. This analysis showed that mature LL-37 is a potent antibacterial peptide...
  89. ncbi Phytochelatin synthase, a dipeptidyltransferase that undergoes multisite acylation with gamma-glutamylcysteine during catalysis: stoichiometric and site-directed mutagenic analysis of arabidopsis thaliana PCS1-catalyzed phytochelatin synthesis
    Olena K Vatamaniuk
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104 6018, USA
    J Biol Chem 279:22449-60. 2004
    ....
  90. ncbi Constitutive expression of cytotoxic proteases and down-regulation of protease inhibitors in LGL leukemia
    Ravi Kothapalli
    H Lee Moffitt Cancer Center and Research Institute, Tampa, FL 33612, USA
    Int J Oncol 22:33-9. 2003
    ..Importantly, the pattern of gene expression in leukemic LGL resembles that seen in activated cytotoxic T cells (CTL)...
  91. ncbi Production of active bovine cathepsin C (dipeptidyl aminopeptidase I) in the methylotrophic yeast Candida boidinii
    T Komeda
    Central Laboratories for Key Technology, Kirin Brewery Co, Ltd, Fukuura, Kanazawa Ku, Yokohama shi, Kanagawa, Japan
    Appl Microbiol Biotechnol 59:252-8. 2002
    ..When the recombinant C. boidinii was grown for 90 h in a jar-fermenter, active CTC was secreted with a yield of up to approximately 12 mg/l...
  92. ncbi Calcium entry into keratinocytes induces exocytosis of lysosomes
    Ralph Jans
    Département Histologie Embryologie, Facultes Universitaires Notre Dame de la Paix, 5000 Namur, Belgium
    Arch Dermatol Res 296:30-41. 2004
    ..These results suggest that upon ionomycin treatment, calcium entry stimulates exocytosis of lysosomes in keratinocytes...
  93. ncbi Synthesis and activity of phosphinic tripeptide inhibitors of cathepsin C
    Artur Mucha
    Institute of Organic Chemistry, Biochemistry and Biotechnology, Wrocław University of Technology, Wybrzeze Wyspianskiego 27, 50 370 Wrocław, Poland
    Bioorg Med Chem Lett 14:3113-6. 2004
    ..However, although designed as transition state analogues, they surprisingly exhibited noncompetitive mode of binding to cathepsin C. Differences in kinetics of C-terminal acids and esters have been additionally observed...
  94. ncbi Leucyl-leucine methyl ester-treated haploidentical donor lymphocyte infusions can mediate graft-versus-leukemia activity with minimal graft-versus-host disease risk
    Michael H Hsieh
    Kimmel Cancer Institute, Jefferson Medical College, Philadelphia, Pennsylvania 19107, USA
    Biol Blood Marrow Transplant 8:303-15. 2002
    L-leucyl-L-leucine methyl ester (LLME) prevents GVHD in several animal models by depleting dipeptidyl peptidase I (DPPI)-expressing cytotoxic cellular subsets...
  95. ncbi Oligomeric structure and substrate induced inhibition of human cathepsin C
    I Dolenc
    Department of Biochemistry and Molecular Biology, J Stefan Institute, Ljubljana, Slovenia
    J Biol Chem 270:21626-31. 1995
    ..2, 6.8, and 7.7 are involved in substrate hydrolysis. Human cathepsin C thus appears to differ qualitatively from other cysteine proteinases of different origin...
  96. ncbi A genetic study of cathepsin C gene in two families with Papillon-Lefèvre syndrome
    Luis M Allende
    Department of Immunology, Hospital 12 de Octubre, Ctra Andalucia, Madrid 28041, Spain
    Mol Genet Metab 79:146-8. 2003
    ..Family 2 shows a previously described non-sense homozygous punctual change (72C>A) that introduces a termination codon at the extracellular domain of the protein (C24X)...
  97. ncbi Dipeptidyl peptidases in human muscle disease
    N C Kar
    Clin Chim Acta 82:185-92. 1978
    ..b>Dipeptidyl peptidase I showed maximum activity at pH 5.0-6.0. Dipeptidyl peptidase II was maximally active at pH 5...
  98. ncbi Cathepsin C and plasma glutamate carboxypeptidase secreted from Fischer rat thyroid cells liberate thyroxin from the N-terminus of thyroglobulin
    Dejan Suban
    Department of Biochemistry and Molecular and Structural Biology, Jozef Stefan Institute, Jamova 39, SI 1000 Ljubljana, Slovenia
    Biochimie 94:719-26. 2012
    ..We focused on the extracellular action of two lysosomal enzymes, cathepsin C (catC, dipeptidyl peptidase I) and PGCP (lysosomal dipeptidase), on thyroglobulin, and their ability to liberate the hormone thyroxin...
  99. ncbi Tetrameric dipeptidyl peptidase I directs substrate specificity by use of the residual pro-part domain
    J G Olsen
    Center for Crystallographic Studies, University of Copenhagen, Denmark
    FEBS Lett 506:201-6. 2001
    The crystal structure of mature dipeptidyl peptidase I reveals insight into the unique tetrameric structure, substrate binding and activation of this atypical papain family peptidase. Each subunit is composed of three peptides...
  100. ncbi Detection of an intragenic deletion expands the spectrum of CTSC mutations in Papillon-Lefèvre syndrome
    Thomas Jouary
    Departement de Dermatologie, Hôpital Saint André, Bordeaux, France
    J Invest Dermatol 128:322-5. 2008
    ..We consider that this technique should be performed in patients with apparently homozygous CTSC mutations when one parent does not carry the expected mutation or is not available for analysis...
  101. ncbi Specific localization of lysosomal aminopeptidases in type II alveolar epithelial cells of the rat lung
    Y Yayoi
    Department of Cell Biology and Neuroscience, Osaka University Graduate School of Medicine, Suita, Japan
    Arch Histol Cytol 64:89-97. 2001
    ....

Research Grants9

  1. Inflammatory Proteases and Cardiac Repair after Myocardial Infarction
    Abdelkarim Sabri; Fiscal Year: 2013
    ..Pilot study shows that NSP deletion in-vivo using DiPeptidyl Peptidase I (DPPI) KO mice, mice that lack major NSPs, attenuated myocyte death following MI and resulted in smaller ..
  2. Mast Cells and the Host Response in the Lung
    Paul Wolters; Fiscal Year: 2007
    ..Recently, we made the discovery that the mast cell protease dipeptidyl peptidase I (DPPI) contributes to death of the host from septic peritonitis and that it appears to do so by regulating ..
  3. LLME TREATED T LYMPHOCYTE THERAPY AFTER ALLOGENEIC BMT
    Neal Flomenberg; Fiscal Year: 2002
    ..The proposed grant will support a trial to administer increasing numbers of LLME-treated DLI to patients and monitor patients for CD4 recovery and other parameters of immune reconstitution as well as for development of GVHD. ..
  4. CYTOTOXIC CELLS AND ALLOIMMUNE RESPONSES
    Dwain Thiele; Fiscal Year: 2002
    ..abstract): The studies detailed in this application will examine the role of the granule thiol protease, dipeptidyl peptidase I (DPPI) in generation of cytotoxic T lymphocyte (CTL) effector functions and the evolution of in vivo ..
  5. MAST CELL CYSTEINE PROTEASES IN LUNG INFLAMMATION
    Paul Wolters; Fiscal Year: 2003
    ..We hypothesize that tissue mast cells are a major source of dipeptidyl peptidase I (DPPI) and a newly identified 60-kDa cysteine protease and that these proteases participate in ..
  6. PULMONARY EMPHYSEMA--ROLE OF SERINE PROTEINASES
    John Hoidal; Fiscal Year: 2000
    ....
  7. DPPI AND CELL MEDIATED IMMUNITY
    Christine Pham; Fiscal Year: 2001
    ..4. We will create a null mutation in the DPPI gene using homologous recombination in embryonic stem cells and analyze the function of immune effector cells derived from these mice. ..
  8. CYTOTOXIC CELLS AND ALLOIMMUNE RESPONSES
    Dwain Thiele; Fiscal Year: 2004
    ..Proposed studies will focus on the role of the granule thiol protease, dipeptidyl peptidase I (DPPI), in both the generation of cytotoxic T lymphocyte (CTL) effector function and in the regulation of ..
  9. Role of DPPI & Serine Proteases in Inflammatory Diseases
    Christine Pham; Fiscal Year: 2007
    ..We also hypothesize that decreased expression of complement regulators in the kidney mav be a determinant that influences the severity of tissue damage in the target organ. ..