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  • 1
    Electronic Resource
    Electronic Resource
    Proteolytic degradation of glutamate decarboxylase mediates disinhibition of hippocampal CA3 pyramidal cells in cathepsin D-deficient mice (2005)
    Oxford, UK : Blackwell Science Ltd
    Journal of neurochemistry 94 (2005), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Although of clinical importance, little is known about the mechanism of seizure in neuronal ceroid lipofuscinosis (NCL). In the present study, we have attempted to elucidate the mechanism underlying the seizure of cathepsin D-deficient (CD–/–) mice that show a novel type of lysosomal storage disease with a phenotype resembling late infantile NCL. In hippocampal slices prepared from CD–/– mice at post-natal day (P)24, spontaneous burst discharges were recorded from CA3 pyramidal cells. At P24, the mean amplitude of IPSPs after stimulation of the mossy fibres was significantly smaller than that of wild-type mice, which was substantiated by the decreased level of γ-aminobutyric acid (GABA) contents in the hippocampus measured by high-performance liquid chromatography (HPLC). At this stage, activated microglia were found to accumulate in the pyramidal cell layer of the hippocampal CA3 subfield of CD–/– mice. However, there was no significant change in the numerical density of GABAergic interneurons in the CA3 subfield of CD–/– mice at P24, estimated by counting the number of glutamate decarboxylase (GAD) 67-immunoreactive somata. In the hippocampus and the cortex of CD–/– mice at P24, some GABAergic interneurons displayed extremely high somatic granular immunoreactivites for GAD67, suggesting the lysosomal accumulation of GAD67. GAD67 levels in axon terminals abutting on to perisomatic regions of hippocampal CA3 pyramidal cells was not significantly changed in CD–/– mice even at P24, whereas the total protein levels of GAD67 in both the hippocampus and the cortex of CD–/– mice after P24 were significantly decreased as a result of degradation. Furthermore, the recombinant human GAD65/67 was rapidly digested by the lysosomal fraction prepared from the whole brain of wild-type and CD–/– mice. These observations strongly suggest that the reduction of GABA contents, presumably because of lysosomal degradation of GAD67 and lysosomal accumulation of its degraded forms, are responsible for the dysfunction of GABAergic interneurons in the hippocampal CA3 subfield of CD–/– mice.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.2005.03250.x
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  • 2
    Electronic Resource
    Electronic Resource
    Proteases involved in MHC dass II antigen presentation (1999)
    Oxford, UK : Blackwell Publishing Ltd
    Immunological reviews 172 (1999), S. 0 
    Details
    ISSN: 1600-065X
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary: Major histocompatibility complex class II antigen presentation requires the participation of lysosomal proteases in two convergent processes. First, the antigens endocytosed by the antigen-presenting cells must be broken down into antigenic peptides. Second, class II tnolecules are synthesized with their peptide-binding site blocked by invariant chain (li), and they acquire the capacity to bind antigens only after Ii has been degraded in the compartments where peptides reside. The study of genetically modified tnice deficietit in single lysosomal proteases has allowed us to determine their role in these processes, Cathepsins (Cat) B and D. previously considered major players in MHC class II antigen presentation, are dispensable for degradation of Ii and for generation of several antigenic determinants. By contrast, Cat S plays an essential role in removal of Ii in B cells and dendritic cells, whereas Cat L apparently does so in thymic epithelial cells. Accordingly, the absence of Cat S and L have major consequences for the onset of humoral immtine responses and for T-cell selection, respectively. It is likely that other as yet uncharacterized lysosomal enzymes also play a role in Ii degradation and in generation of antigenic determinants. Experiments involving drugs that interfere with protein traffic suggest that more than one mechanism for Ii removal, probably involving different proteases, can co-exist in the same antigen-presenting cell. These findings may allow the development of protease inhibitors with possible therapeutic applications.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-065X.1999.tb01360.x
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  • 3
    Electronic Resource
    Electronic Resource
    Genome-wide, large-scale production of mutant mice by ENU mutagenesis (2000)
    [s.l.] : Nature America Inc.
    Nature genetics 25 (2000), S. 444-447 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] In the post-genome era, the mouse will have a major role as a model system for functional genome analysis. This requires a large number of mutants similar to the collections available from other model organisms such as Drosophila melanogaster and Caenorhabditis elegans. Here we report on a ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/78146
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  • 4
    Electronic Resource
    Electronic Resource
    Cathepsin L is required for endothelial progenitor cell–induced neovascularization (2005)
    [s.l.] : Nature Publishing Group
    Nature medicine 11 (2005), S. 206-213 
    Details
    ISSN: 1546-170X
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Infusion of endothelial progenitor cells (EPC), but not of mature endothelial cells, promotes neovascularization after ischemia. We performed gene expression profiling of EPC and endothelial cells to identify genes that might be important for the neovascularization capacity of EPC. Notably, the ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/nm1182
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  • 5
    Electronic Resource
    Electronic Resource
    Therapiekonzept zur kombinierten kieferorthopädisch-kieferchirurgischen Behandlung von Klasse-II-Dysgnathien mit Short-face-Syndrom (2000)
    Springer
    Mund-, Kiefer- und Gesichtschirurgie 4 (2000), S. 118-124 
    Details
    ISSN: 1434-3940
    Keywords: Schlüsselwörter Kephalometrie ; Skelettal tiefer Biss ; Short-face-Syndrom ; Untergesichtsverlängerung ; Aufbissschiene ; Lateral offener Biss ; Key words Cephalometry ; Skeletal deep bite ; Short face syndrome ; Lengthening of short face ; Bite brace ; Lateral open bite
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The main aesthetic concern of patients with Angle class II deformities with skeletal deep bite – short face syndrome – is the short lower face. It is the orthodontist’s task to correct this deformity insofar as possible. Depending on the extent, even orthodontists at the beginning of their training will recognize this. Since presurgical orthodontic treatment determines the kind and extent of the surgical procedure, the orthodontist has to plan the treatment in for individual case . It is the purpose of this article to demonstrate a concept of treatment for patients with class II deformities, skeletal deep bite, and a short lower face. Presurgical orthodontic treatment and the surgical procedure to correct the deformity are discussed. The treatment results show that it is necessary to leave or to create a certain curve of Spee, depending on the extent of the deformity, to achieve a satisfactory result in terms of to function, aesthetics, and stability. It can be concluded that it is only possible to reach the preset treatment goals with an exact diagnosis and the knowledge of the necessary preparation in combination with the surgical procedure.
    Notes: Zusammenfassung Das Hauptproblem einer Angle-Klasse-II-Dysgnathie mit skelettal tiefem Biss (Short-face-Syndrom) stellt für die betroffenen Patienten das verkürzte Untergesicht als ästhetische Beeinträchtigung dar. Für den Kieferorthopäden stellt sich die Aufgabe, dieses je nach Ausprägung auch für den Ungeübten gut erkennbare Defizit des Untergesichts möglichst optimal zu verbessern. Da die orthodontische Vorbereitung die Art und das Ausmaß des chirurgischen Eingriffs wesentlichen beeinflusst, muss der Kieferorthopäde in der prächirurgischen Phase ein fallbezogen geplantes Vorgehen wählen. Im vorliegenden Artikel wird ein Behandlungskonzept zur Therapie von Patienten mit Klasse-II-Dysgnathien, skelettal tiefem Biss und kurzem Untergesicht dargestellt. Dabei wird sowohl auf die kieferorthopädische Vorbereitung als auch auf die chirurgische Korrektur der Dysgnathie eingegangen. Das Behandlungsergebnis zeigt, dass präoperativ je nach Ausprägung der Dysgnathie eine unterschiedlich starke Spee-Kurve belassen bzw. geschaffen werden muss, um aus funktioneller, ästhetischer und stabilitätsbezogener Sicht ein möglichst optimales Ergebnis zu erzielen. Folglich führt nur eine exakte Diagnose in Kenntnis der notwendigen, orthodontischen Vorbereitung in Verbindung mit der dadurch vorgegebenen Dysgnathieoperation zur Erfüllung der angestrebten Behandlungsziele.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s100060050182
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  • 6
    Electronic Resource
    Electronic Resource
    Four novel mutant alleles of the arylsulfatase B gene in two patients with intermediate form of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) (1994)
    Springer
    Human genetics 〈Berlin〉 93 (1994), S. 259-264 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Mucopolysaccharidosis type VI (MPSVI, Maroteaux-Lamy syndrome) is a lysosomal storage disease for which multiple clinical phenotypes have been described. A deficiency of the enzyme arylsulfatase B (ASB, N-acetylgalactosamine-4-sulfatase) is the cause of this autosomal recessively inherited disorder. The genotypes of two patients with an intermediate form of MPSVI have been determined by polymerase chain reaction (PCR) amplification of the entire open reading frame of the ASB gene and subsequent direct sequencing of both strands of the PCR fragments by an automated nonradioactive approach. In patient A, a C to T transition in allele I resulting in an exchange of the Arg codon 160 for a premature stop codon (R160*, exon 2), and a G to A transition in allele II leading to a Gln to Arg160 substitution (R160Q, exon 2) were detected. Patient B exhibited a 7-bp deletion in exon 1 of allele I resulting in a frame shift and a premature stop codon 33 triplets 3′ of the site of deletion (ΔG237-C243), and a C to T transition in allele II giving rise to a Trp to Arg152 substitution (R152W, exon 2). None of these four mutant alleles was present among 60 alleles of the ASB gene in unrelated controls, indicating that the former are not polymorphisms. These results emphasize the broad molecular heterogeneity of Maroteaux-Lamy syndrome and contribute to the establishment of a genotype/phenotype correlation in this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00212019
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  • 7
    Electronic Resource
    Electronic Resource
    The genes of the lysosomal cysteine proteinases cathepsin B, H, L, and S map to different mouse chromosomes (1997)
    Springer
    Mammalian genome 8 (1997), S. 241 -245 
    Details
    ISSN: 1432-1777
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. The mouse genes for the lysosomal cysteine proteinases cathepsin B, H, L, and S were mapped to Chromosomes (Chrs) 14, 9, 13, and 3, respectively. Two of the DNA probes used in this study detected an additional, independently segregating locus. The cathepsin B-specific probe hybridized to a locus on Chr 2, and the cathepsin H probe to a locus on the X Chr. These loci either correspond to pseudogenes or to cathepsin B- and cathepsin H-related genes. The four cysteine proteinases mapped in this study lie within known regions of conserved synteny between mouse and human chromosomes, when compared with the corresponding positions of their human homologs. Assuming that the genes of the cysteine proteinase gene family arose from a common ancestral gene, our results suggest that these four cysteine proteinases had been dispersed over different chromosomes before separation of mouse and human in evolution.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003359900401
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