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Transgene Maus- und molekulare Zellbiologie

	2019 A mouse model for SPG48 reveals a block of autophagic flux upon disruption of adaptor protein complex five Aß34 is a BACE1-derived degradation intermediate associated with amyloid clearance and Alzheimer's disease progression Drug-induced increase in lysobisphosphatidic acid reduces the cholesterol overload in Niemann-Pick type C cells and mice Genetic LAMP2 deficiency accelerates the age-associated formation of basal laminar deposits in the retina Inhibiting pathologically active ADAM10 rescues synaptic and cognitive decline in Huntington's disease Lysosomal degradation of newly formed insulin granules contributes to ß cell failure in diabetes Lysosomal integral membrane protein-2 (LIMP-2/SCARB2) is involved in lysosomal cholesterol export Lysosomal storage disorders - challenges, concepts and avenues for therapy: beyond rare diseases Meprin ß induces activities of A disintegrin and metalloproteinases 9, 10, and 17 by specific prodomain cleavage Presynaptic Endosomal Cathepsin D Regulates the Biogenesis of GABAergic Synaptic Vesicles The intramembrane protease SPPL2c promotes male germ cell development by cleaving phospholamban The lysosomal transporter MFSD1 is essential for liver homeostasis and critically depends on its accessory subunit GLMP Ubiquitin C-terminal hydrolase L1 (UCH-L1) loss causes neurodegeneration by altering protein turnover in the first postnatal weeks
	2018 A homozygous founder missense variant in arylsulfatase G abolishes its enzymatic activity causing atypical Usher syndrome in humans Altered distribution of ATG9A and accumulation of axonal aggregates in neurons from a mouse model of AP-4 deficiency syndrome In vivo regulation of the A disintegrin and metalloproteinase 10 (ADAM10) by the tetraspanin 15. PLD3 and PLD4 are single-stranded acid exonucleases that regulate endosomal nucleic-acid sensing PLD3 and spinocerebellar ataxia RNase H2 Loss in Murine Astrocytes Results in Cellular Defects Reminiscent of Nucleic Acid-Mediated Autoinflammation Structural and mechanistic aspects influencing the ADAM10-mediated shedding of the prion protein The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover Unconventional Trafficking of Mammalian Phospholipase D3 to Lysosomes
	2017 Absence of RNase H2 triggers generation of immunogenic micronuclei removed by autophagy Disruption of the vacuolar-type H+-ATPase complex in liver causes MTORC1-independent accumulation of autophagic vacuoles and lysosomes Diverse functions of the prion protein - Does proteolytic processing hold the key? Functional characterization of the lysosomal membrane protein TMEM192 in mice Haplodeficiency of Cathepsin D does not affect cerebral amyloidosis and autophagy in APP/PS1 transgenic mice Intermittent fasting preserves beta-cell mass in obesity-induced diabetes via the autophagy-lysosome pathway Long-term enzyme replacement therapy improves neurocognitive functioning and hippocampal synaptic plasticity in immune-tolerant alpha-mannosidosis mice Lysosomal integral membrane protein-2 as a phospholipid receptor revealed by biophysical and cellular studies Plekhg5-regulated autophagy of synaptic vesicles reveals a pathogenic mechanism in motoneuron disease Progranulin functions as a cathepsin D chaperone to stimulate axonal outgrowth in vivo Quantitative Proteome Analysis of Mouse Liver Lysosomes Provides Evidence for Mannose 6-phosphate-independent Targeting Mechanisms of Acid Hydrolases in Mucolipidosis II. Sequestration of cholesterol within the host late endocytic pathway restricts liver-stage Plasmodium development Serum Starvation Induces BACE1 Processing and Secretion Tetraspanin 3: A central endocytic membrane component regulating the expression of ADAM10, presenilin and the amyloid precursor protein Tetraspanin CD63 controls basolateral sorting of organic cation transporter 2 in renal proximal tubules The Influence of MHC Class II on B Cell Defects Induced by Invariant Chain/CD74 N-Terminal Fragments The metalloproteinase ADAM10: A useful therapeutic target?
	2016 A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease Age-dependent development of liver fibrosis in Glmp (gt/gt) mice Cathepsin K Deficiency Suppresses Disuse-Induced Bone Loss Characterization of the complex formed by ß-glucocerebrosidase and the lysosomal integral membrane protein type-2 Degeneration of Photoreceptor Cells in Arylsulfatase G-Deficient Mice Dissecting the role of ADAM10 as a mediator of Staphylococcus aureus a-toxin action Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) Impaired Lysosomal Integral Membrane Protein 2-dependent Peroxiredoxin 6 Delivery to Lamellar Bodies Accounts for Altered Alveolar Phospholipid Content in Adaptor Protein-3-deficient pearl Mice Intramembrane proteolysis within lysosomes LAMP proteins account for the maturation delay during the establishment of the Coxiella burnetii-containing vacuole Pancreatic Premalignant Lesions Secrete Tissue Inhibitor of Metalloproteinases-1, Which Activates Hepatic Stellate Cells Via CD63 Signaling to Create a Premetastatic Niche in the Liver Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance SCARB2/LIMP2 deficiency in action myoclonus-renal failure syndrome Substrate determinants of signal peptide peptidase-like 2a (SPPL2a)-mediated intramembrane proteolysis of the invariant chain CD74 Systematic substrate identification indicates a central role for the metalloprotease ADAM10 in axon targeting and synapse function The Emerging Role of Tetraspanins in the Proteolytic Processing of the Amyloid Precursor Protein Turn up the lysosome
	2015 A Cell-Based Assay Reveals Nuclear Translocation of Intracellular Domains Released by SPPL Proteases ADAM17 controls IL-6 signaling by cleavage of the murine IL-6Ra from the cell surface of leukocytes during inflammatory responses Ataxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III) Autophagy in neuronal cells: general principles and physiological and pathological functions Cathepsin D deficiency induces oxidative damage in brain pericytes and impairs the blood-brain barrier Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11 Lack of the Lysosomal Membrane Protein, GLMP, in Mice Results in Metabolic Dysregulation in Liver LAMP-2 deficiency leads to hippocampal dysfunction but normal clearance of neuronal substrates of chaperone-mediated autophagy in a mouse model for Danon disease Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease Mannose 6-phosphate-independent Lysosomal Sorting of LIMP-2 Processing of CD74 by the Intramembrane Protease SPPL2a Is Critical for B Cell Receptor Signaling in Transitional B Cells Proteases at work: cues for understanding neural development and degeneration Repetitive stimulation of autophagy-lysosome machinery by intermittent fasting preconditions the myocardium to ischemia-reperfusion injury ß-Secretase BACE1 regulates hippocampal and reconstituted M-currents in a ß-subunit-like fashion The alpha secretase ADAM10: A metalloprotease with multiple functions in the brain The sheddase ADAM10 is a potent modulator of prion disease TIMP-1 signaling via CD63 triggers granulopoiesis and neutrophilia in mice Vacuolar ATPase in phagosome-lysosome fusion
	2014 ADAM metalloproteases promote a developmental switch in responsiveness to the axonal repellant Sema3A Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis Gene disruption of Mfsd8 in mice provides the first animal model for CLN7 disease High susceptibility to fatty liver disease in two-pore channel 2-deficient mice Leukocytes require ADAM10 but not ADAM17 for their migration and inflammatory recruitment into the alveolar space LIMP-2 expression is critical for ß-glucocerebrosidase activity and a-synuclein clearance. Loss of lysosomal membrane protein NCU-G1 in mice results in spontaneous liver fibrosis with accumulation of lipofuscin and iron in Kupffer cells Molecular characterization of arylsulfatase G: expression, processing, glycosylation, transport, and activity Regulated proteolysis of NOTCH2 and NOTCH3 receptors by ADAM10 and presenilins Signal-peptide-peptidase-like 2a is required for CD74 intramembrane proteolysis in human B cells The endolysosomal cysteine cathepsins L and K are involved in macrophage-mediated clearance of Staphylococcus aureus and the concomitant cytokine induction The intramembrane proteases signal Peptide peptidase-like 2a and 2b have distinct functions in vivo Virus entry. Lassa virus entry requires a trigger-induced receptor switch
	2013 Bace1 and Neuregulin-1 cooperate to control formation and maintenance of muscle spindles Cancer: Killing from the inside. Cathepsin F mutations cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis Cellular mechanotransduction relies on tension-induced and chaperone-assisted autophagy Differences in expression patterns of cathepsin C/dipeptidyl peptidase I in normal, pathological and aged mouse central nervous system Enzyme replacement therapy for alpha-mannosidosis: 12 months follow-up of a single centre, randomised, multiple dose study Epidermal ADAM17 is dispensable for notch activation Extracellular cathepsin K exerts antimicrobial activity and is protective against chronic intestinal inflammation in mice Lysosomal membrane proteins and their central role in physiology Natural history of alpha mannosidosis a longitudinal study Parallel regulation of renin and lysosomal integral membrane protein 2 in renin-producing cells: further evidence for a lysosomal nature of renin secretory vesicles Postnatal disruption of the disintegrin/metalloproteinase ADAM10 in brain causes epileptic seizures, learning deficits, altered spine morphology, and defective synaptic functions Regulation of adult hematopoiesis by the a disintegrin and metalloproteinase 10 (ADAM10) Structure of LIMP-2 provides functional insights with implications for SR-BI and CD36 Surface CD107a/LAMP-1 protects natural killer cells from degranulation-associated damage The intramembrane protease SPPL2A is critical for tooth enamel formation The intramembrane protease SPPL2a promotes B cell development and controls endosomal traffic by cleavage of the invariant chain The tetraspanin CD63 is required for efficient IgE-mediated mast cell degranulation and anaphylaxis
	2012 A critical histidine residue within LIMP-2 mediates pH sensitive binding to its ligand ß-glucocerebrosidase. Activity-dependent proteolytic cleavage of neuroligin-1 Adam10 haploinsufficiency causes freckle-like macules in Hairless mice Arylsulfatase G inactivation causes loss of heparan sulfate 3-O-sulfatase activity and mucopolysaccharidosis in mice Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis Ectodomain shedding and ADAMs in development Effects of cathepsin K deficiency on intercellular junction proteins, luminal mucus layers, and extracellular matrix constituents in the mouse colon Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice Mannose 6 dephosphorylation of lysosomal proteins mediated by acid phosphatases Acp2 and Acp5 Notch-RBP-J signaling regulates the transcription factor IRF8 to promote inflammatory macrophage polarization Physiological functions of the amyloid precursor protein secretases ADAM10, BACE1, and presenilin Sensitivity to lysosome-dependent cell death is directly regulated by lysosomal cholesterol content ß-Secretase (BACE1) inhibition causes retinal pathology by vascular dysregulation and accumulation of age pigment Tetraspanin15 regulates cellular trafficking and activity of the ectodomain sheddase ADAM10 The lysosomal polypeptide transporter TAPL is stabilized by interaction with LAMP-1 and LAMP-2
	2011 Acid ß-glucosidase mutants linked to Gaucher disease, Parkinson disease, and Lewy body dementia alter a-synuclein processing Cathepsin K deficiency in mice induces structural and metabolic changes in the central nervous system that are associated with learning and memory deficits CD63 is an essential cofactor to leukocyte recruitment by endothelial P-selectin Cerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in a-mannosidosis Deletion of Adam10 in endothelial cells leads to defects in organ-specific vascular structures Disrupted in renal carcinoma 2 (DIRC2), a novel transporter of the lysosomal membrane, is proteolytically processed by cathepsin L Lack of a-disintegrin-and-metalloproteinase ADAM10 leads to intracellular accumulation and loss of shedding of the cellular prion protein in vivo LIMP-2 links late phagosomal trafficking with the onset of the innate immune response to Listeria monocytogenes: a role in macrophage activation Neuronal brain-derived neurotrophic factor is synthesized in excess, with levels regulated by sortilin-mediated trafficking and lysosomal degradation Redundancy and specificity of the metalloprotease system mediating oncogenic NOTCH1 activation in T-ALL Role for LAMP-2 in endosomal cholesterol transport Signal-peptide-peptidase-like 2a (SPPL2a) is targeted to lysosomes/late endosomes by a tyrosine motif in its C-terminal tail Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation ß-Site APP-cleaving enzyme 1 (BACE1) cleaves cerebellar Na+ channel ß4-subunit and promotes Purkinje cell firing by slowing the decay of resurgent Na+ current The "A Disintegrin And Metalloproteases" ADAM10 and ADAM17: novel drug targets with therapeutic potential? The disintegrin/metalloproteinase Adam10 is essential for epidermal integrity and Notch-mediated signaling The effect of cathepsin K deficiency on airway development and TGF-ß1 degradation The Swedish APP mutation alters the effect of genetically reduced BACE1 expression on the APP processing The tetraspanin CD63 regulates ESCRT-independent and -dependent endosomal sorting during melanogenesis Tubular proteinuria in mice and humans lacking the intrinsic lysosomal protein SCARB2/Limp-2 Two dileucine motifs mediate late endosomal/lysosomal targeting of transmembrane protein 192 (TMEM192) and a C-terminal cysteine residue is responsible for disulfide bond formation in TMEM192 homodimers
	2010 Activity-dependent alpha-cleavage of nectin-1 is mediated by a disintegrin and metalloprotease 10 (ADAM10) ADAM17 is regulated by a rapid and reversible mechanism that controls access to its catalytic site Cathepsin B-mediated autophagy flux facilitates the anthrax toxin receptor 2-mediated delivery of anthrax lethal factor into the cytoplasm. Cathepsin K gene disruption does not affect murine aneurysm formation Chaperone-assisted selective autophagy is essential for muscle maintenance CNS-expressed cathepsin D prevents lymphopenia in a murine model of congenital neuronal ceroid lipofuscinosis Disease-causing mutations within the lysosomal integral membrane protein type 2 (LIMP-2) reveal the nature of binding to its ligand beta-glucocerebrosidase. Impaired lysosomal trimming of N-linked oligosaccharides leads to hyperglycosylation of native lysosomal proteins in mice with alpha-mannosidosis Lysosomal membrane proteins: life between acid and neutral conditions Molecular characterisation of 'transmembrane protein 192' (TMEM192), a novel protein of the lysosomal membrane Molecular insights into mechanisms of intramembrane proteolysis through signal peptide peptidase (SPP). Stimulation of platelet-derived growth factor receptor beta (PDGFRbeta) activates ADAM17 and promotes metalloproteinase-dependent cross-talk between the PDGFRbeta and epidermal growth factor receptor (EGFR) signaling pathways The disintegrin/metalloproteinase ADAM10 is essential for the establishment of the brain cortex. The proteome of lysosomes
	2009 ADAM10, the rate-limiting protease of regulated intramembrane proteolysis of Notch and other proteins, is processed by ADAMS-9, ADAMS-15, and the gamma-secretase. ADAMs 10 and 17 represent differentially regulated components of a general shedding machinery for membrane proteins such as transforming growth factor alpha, L-selectin, and tumor necrosis factor alpha. Alcadein cleavages by amyloid beta-precursor protein (APP) alpha- and gamma-secretases generate small peptides, p3-Alcs, indicating Alzheimer disease-related gamma-secretase dysfunction. Autophagy: a lysosomal degradation pathway with a central role in health and disease. Bone density, strength, and formation in adult cathepsin K (-/-) mice. Cathepsin D expression level affects alpha-synuclein processing, aggregation, and toxicity in vivo. Cathepsin D is the primary protease for the generation of adenohypophyseal vasoinhibins: cleavage occurs within the prolactin secretory granules. Cathepsin K activity-dependent regulation of osteoclast actin ring formation and bone resorption. Cytoplasmic relaxation of active Eph controls ephrin shedding by ADAM10. Deficiency of the tetraspanin CD63 associated with kidney pathology but normal lysosomal function. Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities. Klotho is a substrate for alpha-, beta- and gamma-secretase. Lysosome biogenesis and lysosomal membrane proteins: trafficking meets function. Molecular characterization and gene disruption of mouse lysosomal putative serine carboxypeptidase 1 NCU-G1 is a highly glycosylated integral membrane protein of the lysosome Non-proteolytic effect of beta-site APP-cleaving enzyme 1 (BACE1) on sodium channel function. Regulated intramembrane proteolysis--a story about sheddases and I-CliPs. The "a disintegrin and metalloprotease" (ADAM) family of sheddases: physiological and cellular functions.
	2008 A soluble form of the receptor for advanced glycation endproducts (RAGE) is produced by proteolytic cleavage of the membrane-bound form by the sheddase a disintegrin and metalloprotease 10 (ADAM10) Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis. ADAM10 regulates endothelial permeability and T-Cell transmigration by proteolysis of vascular endothelial cadherin. ADAM10-mediated E-cadherin release is regulated by proinflammatory cytokines and modulates keratinocyte cohesion in eczematous dermatitis. Array-based gene discovery with three unrelated subjects shows SCARB2/LIMP-2 deficiency causes myoclonus epilepsy and glomerulosclerosis. Cathepsin K deficiency partially inhibits, but does not prevent, bone destruction in human tumor necrosis factor-transgenic mice. Cathepsin K-dependent toll-like receptor 9 signaling revealed in experimental arthritis. Impaired phagosomal maturation in neutrophils leads to periodontitis in lysosomal-associated membrane protein-2 knockout mice. LAMP-2: a control step for phagosome and autophagosome maturation. Live longer with LAMP-2. Lysosomal myopathies: an excessive build-up in autophagosomes is too much to handle Regulated intramembrane proteolysis of Bri2 (Itm2b) by ADAM10 and SPPL2a/SPPL2b Reversal of peripheral and central neural storage and ataxia after recombinant enzyme replacement therapy in alpha-mannosidosis mice Role of cathepsin K in structural changes in brachiocephalic artery during progression of atherosclerosis in apoE-deficient mice. Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: a model of human congenital neuronal ceroid-lipofuscinosis.
	2007 ADAM10 inhibition of human CD30 shedding increases specificity of targeted immunotherapy in vitro ADAM10 regulates FasL cell surface expression and modulates FasL-induced cytotoxicity and activation-induced cell death Altered regulation of phosphatidylinositol 3-kinase signaling in cathepsin D-deficient brain Arrested maturation of Neisseria-containing phagosomes in the absence of the lysosome-associated membrane proteins, LAMP-1 and LAMP-2 Cathepsin D deficiency induces persistent neurodegeneration in the absence of Bax-dependent apoptosis Cathepsin K null mice show reduced adiposity during the rapid accumulation of fat stores Haematopoietic development and immunological function in the absence of cathepsin D Involvement of lysosomal storage-induced p38 MAP kinase activation in the overproduction of nitric oxide by microglia in cathepsin D-deficient mice LAMP proteins are required for fusion of lysosomes with phagosomes LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of beta-glucocerebrosidase Lysosomal integral membrane protein 2 is a novel component of the cardiac intercalated disc and vital for load-induced cardiac myocyte hypertrophy Metalloproteases regulate T-cell proliferation and effector function via LAG-3 Regulated shedding of transmembrane chemokines by the disintegrin and metalloproteinase 10 facilitates detachment of adherent leukocytes Substrate selectivity of epidermal growth factor-receptor ligand sheddases and their regulation by phorbol esters and calcium influx Toll-like receptor 4-dependent contribution of the immune system to anticancer chemotherapy and radiotherapy
	2006 ADAM10 is a principal 'sheddase' of the low-affinity immunoglobulin E receptor CD23 Breaking up the tie: disintegrin-like metalloproteinases as regulators of cell migration in inflammation and invasion 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 activity Cathepsin D deficiency is associated with a human neurodegenerative disorder Cathepsin protease activity modulates amyloid load in extracerebral amyloidosis Control of peripheral nerve myelination by the beta-secretase BACE1 Cutting edge: a novel nonoxidative phagosomal mechanism exerted by cathepsin-D controls Listeria monocytogenes intracellular growth Deafness in LIMP2-deficient mice due to early loss of the potassium channel KCNQ1/KCNE1 in marginal cells of the stria vascularis Disruption of the cathepsin K gene reduces atherosclerosis progression and induces plaque fibrosis but accelerates macrophage foam cell formation Furin-, ADAM 10-, and gamma-secretase-mediated cleavage of a receptor tyrosine phosphatase and regulation of beta-catenin's transcriptional activity Gene profiling of cathepsin K deficiency in atherogenesis: profibrotic but lipogenic LAMP-2 deficient mice show depressed cardiac contractile function without significant changes in calcium handling Multivariate neurocognitive and emotional profile of a mannosidosis murine model for therapy assessment Osteoclastic bone degradation and the role of different cysteine proteinases and matrix metalloproteinases: differences between calvaria and long bone Proteomic identification of desmoglein 2 and activated leukocyte cell adhesion molecule as substrates of ADAM17 and ADAM10 by difference gel electrophoresis Regulated ADAM10-dependent ectodomain shedding of gamma-protocadherin C3 modulates cell-cell adhesion Structural determinants of M-type KCNQ (Kv7) K+ channel assembly
	2005 A role for cathepsin E in the processing of mast-cell carboxypeptidase A ADAM10 cleavage of N-cadherin and regulation of cell-cell adhesion and beta-catenin nuclear signalling ADAM10 mediates E-cadherin shedding and regulates epithelial cell-cell adhesion, migration, and beta-catenin translocation Autophagic vacuoles with sarcolemmal features delineate Danon disease and related myopathies beta Subunits of voltage-gated sodium channels are novel substrates of beta-site amyloid precursor protein-cleaving enzyme (BACE1) and gamma-secretase Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation Neurocognitive and psychotiform behavioral alterations and enhanced hippocampal long-term potentiation in transgenic mice displaying neuropathological features of human alpha-mannosidosis Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease) Phenotypic and biochemical analyses of BACE1- and BACE2-deficient mice Proteolytic degradation of glutamate decarboxylase mediates disinhibition of hippocampal CA3 pyramidal cells in cathepsin D-deficient mice Soluble Axl is generated by ADAM10-dependent cleavage and associates with Gas6 in mouse serum The apoptosis/autophagy paradox: autophagic vacuolization before apoptotic death The NH(2)-terminal transmembrane and lumenal domains of LGP85 are needed for the formation of enlarged endosomes/lysosomes Unifying nomenclature for the isoforms of the lysosomal membrane protein LAMP-2
	2004 Altered morphological and electrophysiological properties of Cajal-Retzius cells in cerebral cortex of embryonic Presenilin-1 knockout mice Cathepsin D is involved in the regulation of transglutaminase 1 and epidermal differentiation Cathepsin D is up-regulated in inflammatory bowel disease macrophages Cathepsin K: a cysteine protease with unique kinin-degrading properties Cathepsin L is involved in cathepsin D processing and regulation of apoptosis in A549 human lung epithelial cells Cell-matrix interaction via CD44 is independently regulated by different metalloproteinases activated in response to extracellular Ca(2+) influx and PKC activation Dimerization of beta-site beta-amyloid precursor protein-cleaving enzyme Distinct roles for ADAM10 and ADAM17 in ectodomain shedding of six EGFR ligands Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts Efficacy of enzyme replacement therapy in alpha-mannosidosis mice: a preclinical animal study Evaluation of the contribution of different ADAMs to tumor necrosis factor alpha (TNFalpha) shedding and of the function of the TNFalpha ectodomain in ensuring selective stimulated shedding by the TNFalpha convertase (TACE/ADAM17) Evidence for a critical role of the tumor necrosis factor alpha convertase (TACE) in ectodomain shedding of the p75 neurotrophin receptor (p75NTR) Partial loss of presenilins causes seborrheic keratosis and autoimmune disease in mice Pivotal role of cathepsin K in lung fibrosis Presenilin 1 mediates the turnover of telencephalin in hippocampal neurons via an autophagic degradative pathway Role for Rab7 in maturation of late autophagic vacuoles
	2003 At the acidic edge: emerging functions for lysosomal membrane proteins Cathepsin K deficiency in pycnodysostosis results in accumulation of non-digested phagocytosed collagen in fibroblasts Deficiency of mannose 6-phosphate receptors and lysosomal storage: a morphometric analysis of hepatocytes of neonatal mice Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice The cell adhesion protein P-selectin glycoprotein ligand-1 is a substrate for the aspartyl protease BACE1 Thyroid functions of mouse cathepsins B, K, and L
	2002 A physiologic signaling role for the gamma -secretase-derived intracellular fragment of APP A role for the lysosomal membrane protein LGP85 in the biogenesis and maintenance of endosomal and lysosomal morphology Decreased intracellular degradation of insulin-like growth factor binding protein-3 in cathepsin L-deficient fibroblasts Involvement of cathepsin E in exogenous antigen processing in primary cultured murine microglia No endogenous A beta production in presenilin-deficient fibroblasts Reduced sperm count and normal fertility in male mice with targeted disruption of the ADP-ribosylation factor-like 4 (Arl4) gene Role of LAMP-2 in lysosome biogenesis and autophagy The bone lining cell: its role in cleaning Howship's lacunae and initiating bone formation The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts
	2001 Biosynthesis of phosphatidylcholine from a phosphocholine precursor pool derived from the late endosomal/lysosomal degradation of sphingomyelin Disease model: LAMP-2 enlightens Danon disease Elevation of beta-amyloid peptide 2-42 in sporadic and familial Alzheimer's disease and its generation in PS1 knockout cells Implication of APP secretases in notch signaling Involvement of nitric oxide released from microglia-macrophages in pathological changes of cathepsin D-deficient mice Overlapping functions of lysosomal acid phosphatase (LAP) and tartrate-resistant acid phosphatase (Acp5) revealed by doubly deficient mice The discrepancy between presenilin subcellular localization and gamma-secretase processing of amyloid precursor protein The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein
	2000 Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons Cathepsin L deficiency as molecular defect of furless: hyperproliferation of keratinocytes and pertubation of hair follicle cycling Ceramide as an activator lipid of cathepsin D Functions of cathepsin K in bone resorption. Lessons from cathepsin K deficient mice mu1A-adaptin-deficient mice: lethality, loss of AP-1 binding and rerouting of mannose 6-phosphate receptors Presenilin function in APP processing Spermatogenesis in mice is not affected by histone H1.1 deficiency Spermatogenesis proceeds normally in mice without linker histone H1t
	1999 A presenilin-1-dependent gamma-secretase-like protease mediates release of Notch intracellular domain Cathepsin D targeted by acid sphingomyelinase-derived ceramide Cell surface presenilin-1 participates in the gamma-secretase-like proteolysis of Notch Early embryonic death of mice deficient in gamma-adaptin Normal lysosomal morphology and function in LAMP-1-deficient mice 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 deficiency Presenilin-1 deficiency leads to loss of Cajal-Retzius neurons and cortical dysplasia similar to human type 2 lissencephaly Proteases involved in MHC class II antigen presentation Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis The function of presenilin-1 in amyloid beta-peptide generation and brain development
	1998 Cathepsins B and D are dispensable for major histocompatibility complex class II-mediated antigen presentation Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein Destabilization of beta-catenin by mutations in presenilin-1 potentiates neuronal apoptosis Downregulation of PS1 expression in neurons decreases beta-amyloid production: a biochemical link between the two major familial Alzheimer's disease genes Impaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient mice Potential role for cathepsin D in p53-dependent tumor suppression and chemosensitivity
	1997 Mice deficient in lysosomal acid phosphatase develop lysosomal storage in the kidney and central nervous system Non-conventional role of lysosomal acid phosphatase in olfactory receptor axons: co-localization with growth-associated phosphoprotein-43
	1996 Amyloidogenic processing of human amyloid precursor protein in hippocampal neurons devoid of cathepsin D Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VI
	1995 Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cells Proteolysis of IGFBPs by cathepsin D in vitro and in cathepsin D-deficient mice
	1994 Mouse cathepsin D gene: molecular organization, characterization of the promoter, and chromosomal localization
	1993 Targeted disruption of the M(r) 46,000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteins



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