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Biomedical subjects

B Franz

Publications and source records attributed to B Franz.

At least 19 recordsLinked to original sources

Paucity of FOXP3+ cells in skin and peripheral blood distinguishes Sézary syndrome from other cutaneous T-cell lymphomas.

Cutaneous T-cell lymphomas (CTCL) are mainly comprised of two variants: mycosis fungoides (MF) with CD4(+) tumor cells confined to the skin and the leukemic Sézary syndrome with tumor cell spread to the blood. In this study, we investigated cutaneous expression of the regulatory T-cell (T(reg)) marker FOXP3 in 30 CTCL patients. Immunohistochemical analysis revealed significantly lower numbers of CD4(+)FOXP3(+) cells within the dermal lymphomononuclear infiltrate of Sézary patients (16% FOXP3(+) cells of CD4(+) cells) in contrast to MF (43% FOXP3(+) cells (P<0.05)) and rare types of CTCL (45% FOXP3(+) cells). Furthermore, CD4(+)FOXP3(+) T cells were also markedly reduced in the CD4(+) population within the peripheral blood of Sézary patients compared to controls as determined by fluorescence-activated cell sorter, quantitative PCR and functional analyses. The data support the conclusion that the neoplastic cells in CTCL do not express the T(reg) marker FOXP3. Our data also identify Sézary syndrome as, to our knowledge, the first reported neoplastic disease with a clear reduction in T(reg) numbers within the CD4(+) population. This lack of T(reg) might account for the more aggressive nature of Sézary syndrome compared with other CTCL.

Adolescent↗

Polymorphisms within the prion-like protein gene (Prnd) and their implications in human prion diseases, Alzheimer's disease and other neurological disorders.

Only 10% of human transmissible spongiform encephalopathies (TSEs) are associated with mutations of the Prnp region encoding the prion protein (PrP). Recently, the murine PrP-like protein doppel (Dpl) was described and was shown to be overexpressed in certain strains of PrP knockout mice and to cause neurological diseases such as ataxia and Purkinje cell loss. To answer the question of whether there are any polymorphisms within the PrP-like protein gene (Prnd) that might cause or be involved in the development of TSEs, we investigated the complete open reading frame of the human Prnd gene from 58 patients who had died of genetic or sporadic Creutzfeldt-Jakob disease (CJD), Alzheimer's disease or other neurological disorders and from 111 controls. We found five new polymorphisms and one frame shift mutation. One silent polymorphism, which does not lead to an altered amino acid sequence, was also observed. Statistical analysis revealed a significant difference in the distribution of the Prnd genotype at codon 174 between sporadic CJD patients and healthy controls.

Alzheimer Disease↗

Spontaneous otoacoustic emissions in neonates and effect of contralateral white noise stimulation.

Neonates younger than the age of 12 weeks (10 full-term, 20 preterm) had an audiological assessment consisting of brainstem audiometry, tympanometry, transiently evoked otoacoustic emissions, and spontaneous otoacoustic emissions with contralateral white noise stimulation. Results from brainstem audiometry, tympanometry, and transiently evoked otoacoustic emissions suggested normal middle ear function and normal cochlear function. All full-term neonates had multiple spontaneous otoacoustic emissions, and contralateral white noise stimulation resulted in enhancement of emissions in 80%, whereas in 20% the emissions were suppressed. In preterm neonates, spontaneous otoacoustic emissions were present in 55%. These emissions were mostly solitary and, in 64%, showed suppression with contralateral white noise stimulation. Embryological data taken into consideration suggest that the pattern of spontaneous otoacoustic emissions in preterm neonates is more likely related to immaturity of the central auditory pathway rather than the cochlea.

Acoustic Stimulation↗

Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals.

Some patients with autoimmune liver disease present with a clinical and/or histological picture showing characteristic findings of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). Various names, mostly overlap syndrome, have been used to describe these cases, which have thus far not been more closely characterized. The aim of this study was the comparison of 20 patients with overlapping features to representative patients considered suffering from typical AIH or typical PBC (20 patients in each group). We found these patients to indeed show a very mixed picture of both conditions biochemically, serologically, and histologically. However, closer analysis suggested that all of these patients were primarily suffering from PBC as all of them had at least either bile duct destruction on histology or anti-M2 positive antimitochondrial antibodies (AMA). We suggest that these PBC patients because of their genetic susceptibility, evidenced by the AIH-characteristic histocompatibility leukocyte antigen (HLA) type B8, DR3, or DR4, developed a more hepatitic picture. Response to immunosuppressive therapy was excellent. We propose that the name "overlap syndrome" be abandoned for "PBC, hepatitic form." These observations not only have pathophysiological implications, but also suggest that therapy of PBC should be guided by the degree of biochemical and histological hepatic involvement.

Adolescent↗

A novel enzyme-linked immunosorbent assay using the recombinant Actinobacillus pleuropneumoniae ApxII antigen for diagnosis of pleuropneumonia in pig herds.

For the surveillance of pig herds infected with porcine pleuropneumonia, an enzyme-linked immunosorbent assay (ELISA) using the recombinant Actinobacillus pleuropneumoniae ApxII protein as species- but not serotype-specific antigen was developed. Using this ELISA, 243 of 400 animals from 22 A. pleuropneumoniae-infected herds were classified as seropositive.

Actinobacillus pleuropneumoniae↗

[Diagnosis of swine dysentery and spirochaetal diarrhea. 2. Effort of macrorestriction analysis for differentiation of intestinal Serpulina relative to determination by culture-biochemical markers following species classification].

Genotypic differentiation by means of macrorestriction fragment profile analysis using Mlul restriction enzyme was carried out differentiating 41 Serpulina field strains from swine (38), dog (2) and a rat as well as ten type and reference strains into 40 electrophoretic types. A dendrogram was created using the average linkage between groups method. At a level of 50% similarity the patterns could be divided into six groups that roughly corresponded to the results yielded by cultural and biochemical methods formerly (FELTRUP et al. 1999). Five of these clusters corresponded to the five known porcine Serpulina species, one cluster contained the S. pilosicoli isolates from dog and rat included in this study. Interestingly all nine investigated indole negative, strongly haemolytic isolates were clustered together in one group with the S. hyodysenteriae strains, so that incidence of indole negative variants of S. hyodysenteriae was confirmed. Because of being grouped together with two S. intermedia isolates, the suitability of B 256 as S. innocens type strain is--in accord to investigations carried out by PETTERSSON et al (1996)--called in question.

Animals↗

Stress electrocochleography.

Electrocochleographic recordings after changes of middle-ear and intracranial pressure were studied in a group of otologically normal subjects and in patients with suspected Ménière's disease. Electrocochleography performed under these conditions was called stress electrocochleography. It was useful to distinguish among Ménière's disease, benign positional vertigo, and a round-window fistula. Changes of middle-ear pressure, whether positive or negative, always resulted in a rise of the SP/AP ratio. Raised intracranial pressure after Trendelenburg positioning showed fluctuations of the SP/AP ratio in both normal subjects and symptomatic patients. However, in normal subjects, a congruent pattern of these fluctuations was apparent, whereas symptomatic patients exhibited a discordant pattern. The discordant pattern, typical for Ménière's disease, showed an increase of the SP voltage, whereas the AP voltage decreased. In benign positional vertigo, the SP voltage decreased, and the AP voltage increased, once during Trendelenburg positioning and again at the end of the test when starting position was resumed. During raised intracranial pressure after the Queckenstedt maneuver, round-window fistulas showed an increase of the SP voltage, whereas the AP voltage decreased considerably. At our clinic, stress electrocochleography has become an important neurootological test in the differential diagnosis of Ménière's disease.

Action Potentials↗

The cervicogenic otoocular syndrome: a suspected forerunner of Ménière's disease.

Over a period of 4 years, 420 patients were observed with fullness in the ear, episodic vertigo, fluctuating hearing, and tinnitus. Of them, 182 patients showed normal hearing levels, a mild eustachian tube dysfunction, normal SP/AP ratios, mydriasis on the side of the affected ear, and a functional disorder of the upper cervical spine. These patients responded to conservative management, particularly physiotherapy. Additionally, 51 patients showed normal hearing levels, a mild eustachian tube dysfunction, an elevated SP/AP ratio suggesting endolymphatic hydrops, mydriasis on the side of the affected ear, and a functional disorder of the upper cervical spine. These patients were given a diagnosis of cervicogenic otoocular (COO) syndrome. Within this group, 43 patients did not respond to preceding conservative management, and 3 patients developed Ménière's disease within 12 months. Also within this group, 8 patients responded to physiotherapy only, with return of the SP/AP ratio to normal levels. A total of 187 patients had a sensorineural hearing loss, an elevated SP/AP ratio, mydriasis on the side of the affected ear, a functional disorder of the upper cervical spine; 186 had a mild eustachian tube dysfunction and had diagnosed Ménière's disease. Patients with a COO syndrome invariably responded to the insertion of a middle-ear ventilation tube, with return of the SP/AP ratio to normal levels and relief of symptoms. Patients with Ménière's disease had a mixed response. The COO syndrome is suspected to be a forerunner of Ménière's disease.

Adult↗

Development of an ELISA technique for serodiagnosis of bovine paratuberculosis.

A local clinical Mycobacterium avium ssp. paratuberculosis (M. paratuberculosis) isolate was cultivated in large-scale culture. A procedure for efficient preparation of a lipoarabinomannan-containing antigen was developed and standardized; 25 mg of purified antigen were obtained per gram of bacterial wet weight. An ELISA based on this antigen was developed. Intra- and interassay variation were determined to be 20% and 27%, respectively. The ELISA was evaluated using the sera of groups of 39 non-randomly selected and 92 randomly selected animals from which ileocaecal lymph nodes were cultured to isolate viable M. paratuberculosis. Combining the results of both groups a positive predictive value of 74% and a negative predictive value of 99% were calculated. The ELISA was licensed by the German regulatory agencies and, in a direct comparison, was found to be superior to both other licensed assays.

Animals↗

Characterization of a large transferrin-binding protein from Actinobacillus pleuropneumoniae serotype 7.

The binding of transferrin at the surface of Actinobacillus pleuropneumoniae (A. pp.) is mediated by two proteins of approximately 60 and 100 kDa. The 60 kDa protein has been shown to be highly divergent among different serotypes and to induce a serotype-specific protective immune response. In this study we have characterized the 100 kDa transferrin-binding protein of A. pp. serotype 7 and designated it as TfbB. The tfbB gene was found to be located immediately downstream of the tfbA gene. It was cloned and sequenced, and antibodies raised against the isolated recombinant protein detected, with a constant intensity, a 100 kDa protein in A. pp. serotypes 2, 4, 6, 7, 8, 9, 10 and 11, and a polypeptide of approximately 103 kDa in serotypes 1, 3, 5A and 12. In addition, comparative analysis of the deduced amino acid sequence showed more than 40% identity with the large transferrin-binding proteins of Neisseria meningitidis and Haemophilus influenzae. The TfbB protein was expressed in E. coli outer membranes in a conformation eliciting porcine transferrin-specific binding activity. Sera of pigs immunized with these TfbB-containing E. coli membranes recognized functional membrane-associated TfbB protein whereas no such reaction was observed upon immunization with isolated recombinant TfbB protein. A preliminary animal experiment showed that TfbB-containing outer membrane preparations from recombinant E. coli can reduce significantly the mortality of an A.pp. infection with the homologous strain.

Actinobacillus pleuropneumoniae↗

Interference of peptides and specific antibodies with the function of the Actinobacillus pleuropneumoniae transferrin-binding protein.

Multiple-antigenic peptides (MAPs) containing transferrin-binding domains of the Actinobacillus pleuropneumoniae serotype 7-derived transferrin-binding protein (TfbA) (K. Strutzberg, L. von Olleschik, B. Franz, C. Pyne, M. A. Schmidt, and G.-F. Gerlach, Infect. Immun. 63:3846-3850, 1995) were constructed. It was found that the MAPs inhibited transferrin binding of the recombinant TfbA protein, whereas antibodies directed against transferrin-binding domains failed to do so.

Actinobacillus pleuropneumoniae↗

[Demonstration of pneumonia in swine as a constant problem: culture and immunofluorescence microscopic studies of bronchioalveolar lavage (BAL) and serological findings].

Bronchoalveolar lavage was performed in 128 pigs from five fattening units showing acute pneumonia (48 animals), subclinical purulent pneumonia (17 animals), and chronic purulent pneumonia (63 animals). These samples were investigated for bacteria. Additionally immunofluorescence microscopy as well as serological investigations were performed to detect antibodies against several bacteria and viruses. Pasteurella multocida could be detected in more than a half of the samples of pigs with acute pneumonia. Bordetella bronchiseptica and mycoplasmas were isolated in a lower amount. Probably these bacteria infected the pigs of at least one herd after a primary infection with influenza virus because (i) influenza virus could be detected in three of four animals investigated for influenza virus by culture methods, (ii) the virus could be detected in one third of the animals investigated for by immunofluorescence microscopy, and (iii) antibodies against influenza virus could be detected in almost all animals. From pigs with subclinical purulent pneumonia Bordetella bronchiseptica as the only bacterial lung pathogen could be isolated exclusively from nearly each sample. From the samples of pig suffering from chronic purulent pneumonia first of all Bordetella bronchiseptica, Pasteurella multocida and different mycoplasma species could be detected. Using cultural methods Actinobacillus pleuropneumoniae could be isolated from six samples only, in contrast to frequent positive reactions against Actinobacillus pleuropneumoniae antigens obtained by immunofluorescence microscopy and CFT.

Actinobacillus Infections↗

Growth hormone secretion timing in depression: clinical outcome comparisons.

Growth hormone (GH) secretion in the 100 minutes preceding sleep onset (preSO), as well as in the first half of the night (1st HN), was examined for a group of 13 healthy women and 43 women with recurrent depression who participated in a 3-year maintenance therapy study. GH studies were obtained at several points during treatment and every 3 months during maintenance, during which patients were randomly assigned to active drug or drug-free maintenance treatment cells for 3 years, or until recurrence of depression. Depressed patients were divided into subgroups according to their maintenance treatment assignment (active drug or drug free) and treatment outcome (completing in remission or having a recurrence). Imipramine caused an increase in the GH ratio in all subgroups. Protocol completers had a significantly larger imipramine-induced increase in the GH ratio than did recurrers. The difference in time of GH secretion relative to sleep onset was found to correlate with treatment outcome and was independent of medication status during maintenance.

Adult↗

The Holliday junction intermediates of lambda integrative and excisive recombination respond differently to the bending proteins integration host factor and excisionase.

In lambda site-specific recombination, the integrative and excisive reactions proceed via two different Holliday junction intermediates, both of which are generated and resolved by a pair of sequentially ordered single strand exchanges. Factors affecting the directionality and efficiency of the second pair of strand exchanges were examined using artificial Holliday junctions (chi-forms). The integrative and excisive recombination intermediates respond differently to the accessory DNA bending proteins integration host factor and excisionase (Xis). These differences between the two recombination intermediates result from a different interaction pattern between proteins binding to the left (P arm) and right (P' arm) of the crossover region. The effect of Xis protein on the directionality of resolution, i.e. the choice of which strands are exchanged, is consistent with a role in promoting the second strand exchange during excision. Proteins binding to the left of the crossover region (P arm) primarily influence the directionality of resolution, while proteins binding to the right (P' arm) have a greater effect on the overall efficiency of resolution. Together, the effect of proteins binding to sites in the P and P' arms is to greatly enhance resolution of the two different Holliday intermediates and to favor resolution in the 'forward' direction for both integrative and excisive recombination.

Bacteriophage lambda↗

Localization of viable bacteria and bacterial antigens in arthritic joints of Erysipelothrix rhusiopathiae-infected pigs.

Chronic polyarthritis was induced in pigs by infection with Erysipelothrix rhusiopathiae (serovar 2, strain T28). Viable bacteria could be reisolated as long as 5 months post-infection from synovial fluid, synovial tissue and from isolated chondrocytes. The number of viable bacteria could be increased by hypotonic shock of the chondrocytes indicating a substantial intracellular amount of bacteria. Bacterial antigens were shown by immunohistochemistry to be present on the surface of both chondrocytes and synovial cells in arthritic joints. Neither viable bacteria nor bacterial antigen were detected in unaffected joints.

Animals↗