Research Topics
Genomes and Genes
| P SaftigSummaryCountry: Germany Publications
| Collaborators
|
Detail Information
Publications
Impaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient miceP Saftig
Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universitat Gottingen, Gosslerstrasse 12 d, 37073 Gottingen, Germany
Proc Natl Acad Sci U S A 95:13453-8. 1998..Assaying the resorptive activity of cathepsin-K-deficient osteoclasts in vitro revealed this function to be severely impaired, which supports the contention that cathepsin K is of major importance in bone remodeling...
Functions of cathepsin K in bone resorption. Lessons from cathepsin K deficient miceP Saftig
Abteilung Biochemie II, Universitat Gottingen, Germany
Adv Exp Med Biol 477:293-303. 2000..Taken together the phenotype of cathepsin K knockout mice underlines the importance of this proteinase in bone remodelling...- Overlapping functions of lysosomal acid phosphatase (LAP) and tartrate-resistant acid phosphatase (Acp5) revealed by doubly deficient miceA Suter
Zentrum Biochemie und Molekulare Zellbiologie, Abt Biochemie II, Universitat Gottingen, Heinrich Düker Weg 12, 37073 Gottingen, Germany
Development 128:4899-910. 2001..We conclude that for several substrates LAP and Acp5 can substitute for each other and that these acid phosphatases are essential for processing of non-collagenous proteins, including osteopontin, by osteoclasts... - Normal lysosomal morphology and function in LAMP-1-deficient miceN Andrejewski
Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universitat Gottingen, 37073 Gottingen, Germany
J Biol Chem 274:12692-701. 1999..The increase of LAMP-2 was neither correlated with an increase in the level of lamp-2 mRNAs nor with increased half-life time of LAMP-2. This findings suggest a translational regulation of LAMP-2 expression... - Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient miceY Tanaka
Zentrum Biochemie und Molekulare Zellbiologie, Abt Biochemie II, Universitat Gottingen, Germany
Nature 406:902-6. 2000..This theory is further substantiated by the finding that human LAMP-2 deficiency causing Danon's disease is associated with the accumulation of autophagic material in striated myocytes... - Disease model: LAMP-2 enlightens Danon diseaseP Saftig
Zentrum Biochemie und Molekulare Zellbiologie, Abt Biochemie II, Universitat Gottingen, Heinrich Düker Weg 12, 37073 Gottingen, Germany
Trends Mol Med 7:37-9. 2001..LAMP-2 deficient mice represent a valuable animal model of Danon disease. They will further be used to study the exact role of LAMP-2 in autophagy and to analyse the consequences of an impaired autophagic pathway in various tissues... - Mice deficient in lysosomal acid phosphatase develop lysosomal storage in the kidney and central nervous systemP Saftig
Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, Universitat Gottingen, Gosslerstrasse 12d, 37073 Gottingen, Federal Republic of Germany
J Biol Chem 272:18628-35. 1997..The causal relationship of the enzyme defect to the clinical manifestations remains to be determined... - Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VIM Evers
Abteilung Biochemie II, Universitat Gottingen, Germany
Proc Natl Acad Sci U S A 93:8214-9. 1996..This mouse model will be a valuable tool for studying pathogenesis of MPS VI and may help to evaluate therapeutical approaches for lysosomal storage diseases... - Spermatogenesis proceeds normally in mice without linker histone H1tB Drabent
Zentrum Biochemie und Molekulare Zellbiologie, Abt Molekularbiologie, Universitat Gottingen, Germany
Histochem Cell Biol 113:433-42. 2000..RNase protection analysis demonstrated that H1.1, H1.2 and H1.4 histone gene expression is enhanced during spermatogenesis in H1t-deficient mice... - Amyloidogenic processing of human amyloid precursor protein in hippocampal neurons devoid of cathepsin DP Saftig
Zentrum Biochemie und Molekular Zellbiologie, Abteilung Biochemie II, Universitat Gottingen, Gosslerstrasse 12d, 37073 Gottingen
J Biol Chem 271:27241-4. 1996..The results rule out cathepsin D as a critical component of alpha-, beta-, or gamma-secretase and therefore as a primary target for drugs aimed at decreasing the betaA4-amyloid peptide burden in Alzheimer's disease... - mu1A-adaptin-deficient mice: lethality, loss of AP-1 binding and rerouting of mannose 6-phosphate receptorsC Meyer
Zentrum für Biochemie und Molekulare Zellbiologie, Dept Biochemie II, Universitat Gottingen, Heinrich Düker Weg 12, D 37073 Gottingen, Germany
EMBO J 19:2193-203. 2000..MPR46 fails to recycle back from the endosome to the TGN, indicating that AP-1 is required for retrograde endosome to TGN transport of the receptor... - Targeted disruption of the M(r) 46,000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteinsA Koster
Universitat Gottingen, Germany
EMBO J 12:5219-23. 1993..The phenotype of MPR46 -/- mice was normal, indicating mechanisms that compensate the MPR46 deficiency in vivo... - Mouse cathepsin D gene: molecular organization, characterization of the promoter, and chromosomal localizationM Hetman
Universitat Gottingen, Germany
DNA Cell Biol 13:419-27. 1994..In contrast to the 5' region of human cathepsin D, the murine gene contains three CCAAT boxes but lacks any of the four AP2 binding sites found in the human gene... - The function of presenilin-1 in amyloid beta-peptide generation and brain developmentP Saftig
Zentrum Biochemie und Molekulare Zellbiologie, Abteilung Biochemie II, , Germany
Eur Arch Psychiatry Clin Neurosci 249:271-9. 1999.... - Early embryonic death of mice deficient in gamma-adaptinD Zizioli
Zentrum für Biochemie und Molekulare Zellbiologie, Dept Biochemie II, Universitat Gottingen, Gosslerstrasse 12d, 37073 Gottingen, Germany
J Biol Chem 274:5385-90. 1999..Free beta1- or micro1 chains were not detectable, indicating that they are unstable unless they are part of AP-1 complexes. Heterozygous mice weigh less then their wild-type littermates and show impaired T cell development... - Spermatogenesis in mice is not affected by histone H1.1 deficiencyS Rabini
Abt Molekularbiologie, Zentrum Biochemie und Molekulare Zellbiologie, Universitat Gottingen, Humboldtallee 23, Gottingen, 37073, Germany
Exp Cell Res 255:114-24. 2000..1 mRNA and H1.1 protein. No anatomic abnormalities could be detected. In addition, mice lacking the H1.1 gene were fertile and they showed normal spermatogenesis and testicular morphology... - Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cellsP Saftig
Abt Biochemie II, Universitat Gottingen, Germany
EMBO J 14:3599-608. 1995.... - Calvarial osteoclasts express a higher level of tartrate-resistant acid phosphatase than long bone osteoclasts and activation does not depend on cathepsin K or L activityS Perez-Amodio
Experimental Periodontology, Academic Center for Dentistry Amsterdam, Universiteit van Amsterdam and Vrije Universiteit, Louwesweg 1, 1066 EA Amsterdam, The Netherlands
Calcif Tissue Int 79:245-54. 2006.... - Gene profiling of cathepsin K deficiency in atherogenesis: profibrotic but lipogenicS P M Lutgens
Departments of Pathology, Cardiovascular Research Institute Maastricht CARIM, University of Maastricht, P Debyelaan 25, Maastricht, The Netherlands
J Pathol 210:334-43. 2006..Besides this profibrotic effect, cathepsin K deficiency has a lipogenic effect owing to increased lipid uptake mediated by CD36 and caveolins... - Disruption of the cathepsin K gene reduces atherosclerosis progression and induces plaque fibrosis but accelerates macrophage foam cell formationE Lutgens
Department of Pathology, Cardiovascular Research Institute Maastricht, University of Maastricht, Maastricht, The Netherlands
Circulation 113:98-107. 2006.... - Cathepsin K deficiency in pycnodysostosis results in accumulation of non-digested phagocytosed collagen in fibroblastsV Everts
Department of Cell Biology and Histology, Academic Medical Centre, University of Amsterdam, 1100 DE Amsterdam, The Netherlands
Calcif Tissue Int 73:380-6. 2003..This may explain why cathepsin K-deficient mice lack the dysostotic features that are prominent in patients with pycnodysostosis... - The bone lining cell: its role in cleaning Howship's lacunae and initiating bone formationV Everts
Department of Cell Biology and Histology, Academic Medical Centre, University of Amsterdam, The Netherlands
J Bone Miner Res 17:77-90. 2002.... - Presenilin 2 deficiency causes a mild pulmonary phenotype and no changes in amyloid precursor protein processing but enhances the embryonic lethal phenotype of presenilin 1 deficiencyA Herreman
Neuronal Cell Biology Laboratory, K U Leuven and Flanders Institute for Biotechnology, 3000 Leuven, Belgium
Proc Natl Acad Sci U S A 96:11872-7. 1999..These results demonstrate in vivo that PS1 and PS2 have partially overlapping functions and that PS1 is essential and PS2 is redundant for normal Notch signaling during mammalian embryological development... - Reduced sperm count and normal fertility in male mice with targeted disruption of the ADP-ribosylation factor-like 4 (Arl4) geneA Schurmann
Institute of Pharmacology, Medical Faculty, Technical University of Aachen, D 52057 Aachen, Germany
Mol Cell Biol 22:2761-8. 2002..It is suggested that the disruption of Arl4 produces a moderate retardation of germ cell development, possibly at the stage of meiosis... - Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophyB Hess
Institut für Biochemie II, Georg August Universitat Gottingen, Federal Republic of Germany
Proc Natl Acad Sci U S A 93:14821-6. 1996..Neurologic examination reveals significant impairment of neuromotor coordination... - Involvement of nitric oxide released from microglia-macrophages in pathological changes of cathepsin D-deficient miceH Nakanishi
Laboratory of Oral Aging Science, Division of Oral Biological Sciences, Faculty of Dental Sciences, Kyushu University, Fukuoka 812 8582, Japan
J Neurosci 21:7526-33. 2001..These results suggest that NO production via iNOS activity in microglia and peripheral macrophages contributes to secondary tissue damages such as neuronal apoptosis and intestinal necrosis, respectively... - Cathepsins B and D are dispensable for major histocompatibility complex class II-mediated antigen presentationJ Deussing
Abteilung Innere Medizin I, Albert Ludwigs Universitat Freiburg, Freiburg, 79106 Germany
Proc Natl Acad Sci U S A 95:4516-21. 1998..Degradation of Ii proceeded normally in Cat B-/- splenocytes, as it did in Cat D-/- cells. We conclude that neither Cat B nor Cat D are essential for MHC class II-mediated antigen presentation... - Cathepsin L deficiency as molecular defect of furless: hyperproliferation of keratinocytes and pertubation of hair follicle cyclingW Roth
Institut für Molekulare Medizin und Zellforschung, Albert Ludwigs Universitat Freiburg, 79106 Freiburg, Germany
FASEB J 14:2075-86. 2000..CTSL is the first lysosomal proteinase shown to be essential for epidermal homeostasis and regular hair follicle morphogenesis and cycling... - Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neuronsM Koike
Department of Cell Biology and Neurosciences, Osaka University Graduate School of Medicine, Suita, Osaka 565 0871, Japan
J Neurosci 20:6898-906. 2000..These results suggest that the CNS neurons in CD-/- mice show a new form of lysosomal accumulation disease with a phenotype resembling neuronal ceroid lipofuscinosis... - Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosisS Stinchi
Dipartimento di Biologia Cellulare e Molecolare, Sezione di Biochimica e Biologia Molecolare, Universita degli Studi di Perugia, Via del Giochetto, 06126 Perugia, Italy
Hum Mol Genet 8:1365-72. 1999..This mouse model will be a valuable tool for studying the pathogenesis of inherited alpha-mannosidosis and may help to evaluate therapeutic approaches for lysosomal storage diseases... - Presenilin-1 deficiency leads to loss of Cajal-Retzius neurons and cortical dysplasia similar to human type 2 lissencephalyD Hartmann
Anatomisches Institut der CAU Kiel, Otto Hahn Platz 8, D 24 118, Kiel, Germany
Curr Biol 9:719-27. 1999..Physiologically, the presenilins are involved in the Notch and Wnt-beta-catenin signaling pathways... - Ceramide as an activator lipid of cathepsin DM Heinrich
Institute of Immunology, University of Kiel, Germany
Adv Exp Med Biol 477:305-15. 2000..Ceramide accumulation in cells derived from A-ceramidase defective Farber patients correlates with enhanced CTSD activity. These findings suggest that A-SMase-derived ceramide targets endolysosomal CTSD... - Cathepsin protease activity modulates amyloid load in extracerebral amyloidosisC Rocken
Institute of Pathology, Charite University Hospital, Berlin, Germany
J Pathol 210:478-87. 2006..CathL was identified as an amyloid-promoting and CathK as an amyloid-retarding cysteine protease. CathB may only modulate the primary structure of the amyloid peptide without affecting amyloid load...