A complete cDNA sequence for ribosomal protein S6 of Xenopus laevis.
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Animals↗
Biomedical subjects
K L Jones
Publications and source records attributed to K L Jones.
Sagittal craniosynostosis, Dandy-Walker malformation, and hydrocephalus: a unique multiple malformation syndrome.
The Dandy-Walker malformation and craniosynostosis have each been described as isolated occurrences and as components of multiple malformation syndromes. The purpose of this report is to delineate the characteristics of a multiple malformation syndrome of Dandy-Walker malformation and sagittal craniosynostosis. The inheritance pattern appears to be autosomal dominant.
Abnormalities, Multiple↗
Extension of the cleft lip phenotype: the subepithelial cleft.
We present a previously undescribed occult malformation of the upper lip manifest by discontinuity of the orbicularis oris muscle. The relationship of this defect to our current concept of abnormal maxillary process development is discussed along with a proposal to extend the cleft lip phenotype.
Cleft Lip↗
Synovial fluid macrophages and blood monocytes differ in their response to IL-4.
IL-4 has been described as a potential anti-inflammatory molecule because of its ability in vitro to down-regulate human monocyte production of proinflammatory mediators. In this study, the activity of IL-4 on mononuclear cells and CD14+ macrophages from a site of chronic inflammation, namely, the joints of patients with rheumatoid and psoriatic arthritis, was investigated and compared directly with its activity on PBMC and monocytes from the same patients. In contrast to the response by blood monocytes, the response to IL-4 by synovial fluid cells was selective; IL-4 did not significantly suppress LPS-induced TNF-alpha production, but decreased CD14 expression to a similar extent in the two cell populations. IL-4 induction of monocyte/macrophage CD23 expression was investigated as a stimulatory response to IL-4, and although significantly increased on synovial fluid CD14+ cells by IL-4, the induction was considerably reduced compared with that measured for blood cells. Activation and differentiation in vitro of blood monocytes reduced their response to IL-4 for decreased TNF-alpha production and induction of CD23 and suggested that these biologic phenomena may contribute to the decreased responsiveness to IL-4 by synovial fluid monocytes/macrophages. Thus, IL-4 does not have the same anti-inflammatory properties in vitro on synovial fluid cells as on blood monocytes.
Antigens, CD↗
Facio-cutaneous-skeletal syndrome is the Costello syndrome.
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Abnormalities, Multiple↗
Cis-urocanic acid stimulates human peripheral blood monocyte prostaglandin E2 production and suppresses indirectly tumor necrosis factor-alpha levels.
Photoisomerization of trans-urocanic acid (UCA) in the stratum corneum has been implicated in the immunosuppression detected after irradiation with UVB (UV wavelength of 280-320 nm). In this study, cis-urocanic acid suppressed human monocyte production of TNF-alpha by a PGE2-dependent mechanism. This contrasted with the mechanism involving histamine type 2 receptors by which the UCA structural analogue, histamine, suppressed monocyte TNF-alpha production. Histamine type 1 receptor antagonists were without effect on both the cis-UCA- and histamine-induced suppression of monocyte TNF-alpha levels. As indomethacin can reverse UVB-immunosuppression in murine models, we may have identified one of the cellular mechanisms responsible for reduced delayed-type hypersensitivity responses. Decreased TNF-alpha levels, by restricting further cytokine recruitment, may also limit the development of the inflammatory components of hypersensitivity responses.
Brompheniramine↗
Mirror hands and feet with a distinct nasal defect, an autosomal dominant condition.
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Abnormalities, Multiple↗
Duplication (20p) in association with thyroid carcinoma.
We report on a girl with short stature, mental retardation, mutism, "coarse" facial appearance, and papillary-follicular thyroid carcinoma. She had dup(20p) derived from a paternal balanced translocation [(12p;20p)]. We speculate that the carcinoma in our patient may be related to the deletion of material from 12p resulting in absence of genetic material normally required for the suppression of thyroid tumorigenesis.
Adenocarcinoma↗
Brugia pahangi: effects of protective resistance on lymphatic lesions and granulomatous inflammation in infected jirds (Meriones unguiculatus).
Effects of protective resistance on lymphatic lesions and granulomatous inflammation in infected jirds (Meriones unguiculatus). Experimental Parasitology 77, 395-404. The hypothesis that protective immune responses play a role in the induction of filarial-associated lymphatic lesions was tested in jirds immunized twice with 75 Brugia pahangi radiation-attenuated third-stage larvae. Lymphatic lesions and granulomatous reactivity were compared in immunized, infected, and naive jirds at both acute and chronic periods following challenge with 100 third-stage larvae. Challenge worm burdens were reduced in immunized jirds at both infection periods. The ratio of lymph thrombi to lymphatic worms, an indicator of lymphatic lesion severity, was significantly greater in immunized jirds than in nonimmunized-challenged jirds during acute but not chronic infections. Parasite-specific-granulomatous hypersensitivity was assessed by measurements of granuloma areas around B. pahangi-soluble adult worm antigen-coated sepharose beads embolized in the lungs prior to necropsy. Marked granulomatous inflammatory responses seen during the acute period in both immunized-challenged and nonimmunized-challenged jirds were significantly reduced in similar jirds during chronic periods. Jirds with existing B. pahangi infections were not resistant to homologous challenge infection and had fewer lymphatic lesions and reduced granulomatous inflammatory responses to soluble adult worm antigen compared to previously naive jirds at acute periods postchallenge. These data suggest that protective immune responses increase the severity of filariae-induced lymphatic inflammation. The subsequent modulation of these lesions is probably associated with parasites that survived the protective immune response.
Animals↗
Characterization of cloned human endothelin receptors.
Two subtypes of human endothelin receptors, ETA and ETB, have been cloned and stably expressed in Chinese Hamster Ovary cells. These receptors have been characterized by [125I]-endothelin-1 binding and phosphatidyl inositol hydrolysis using the potent peptidyl ETA antagonists BQ-123 and BQ-153, as well as the potent ETB agonist, sarafotoxin S6c. In binding studies, Ki values for BQ-123 and BQ-153 are 17 nM and 13 nM for ETA compared to 11,100 nM and 7200 nM for ETB. Conversely, Ki values for sarafotoxin S6c are 2800 nM for ETA and 0.29 nM for ETB. Endothelin-1 stimulates phosphatidyl inositol hydrolysis in cells expressing either ETA or ETB with EC50 values of 0.2-0.3 nM, while sarafotoxin S6c stimulates phosphatidyl inositol hydrolysis only in ETB expressing cells with an EC50 value of 0.2 nM, consistent with the binding data. Comparison of binding data for the cloned and expressed human receptors with binding data for receptors obtained from human tissues indicates the cloned and expressed receptors are essentially indistinguishable from the naturally occurring receptors.
Animals↗
Neonatal hypoglycemia caused by hypopituitarism in infants with congenital syphilis.
Two infants with congenital syphilis and persistent hypoglycemia were found to have hypopituitarism. Hypopituitarism should be recognized as a potential complication of congenital syphilis; affected infants with persistent hypoglycemia should receive a prompt evaluation of pituitary function.
Humans↗
Reduced secretion of IL-1 beta by peritoneal cells from patients on continuous ambulatory peritoneal dialysis.
The endogenous and lipopolysaccharide stimulated interleukin (IL)-1 beta production in vitro by peritoneal monocytes/macrophages from patients on continuous ambulatory peritoneal dialysis (CAPD) was examined during episodes of infection and inflammation. Measurement of immunoreactive IL-1 beta and bioactive IL-1 in both supernatants and cell lysates after culture for 18 h revealed that these cells secreted a significantly lower proportion of total IL-1 than that measured for elutriated blood monocytes. For the inflammatory peritoneal cells, the proportion of total IL-1 beta that was cell-associated resembled that reported for more differentiated pulmonary alveolar macrophages and for adherent monocytes cultured for 18 h prior to stimulation. A similar reduced ability to secrete IL-1 beta was detected for unfractionated peritoneal cells from CAPD patients without peritonitis upon direct comparison with the IL-1 beta production by blood mononuclear cells from the same patients. These results suggested that at a time when a pro-inflammatory response by extravasated host monocytes/macrophages was required by CAPD patients with peritonitis, only a minor proportion of total IL-1 beta would be available extracellularly. This study highlights the rapidity with which extravasated monocytes lose their ability to secrete IL-1 beta and raises the possibility that an important site of utilization of IL-1 beta in vivo may be intracellular in its location.
Humans↗
Mapping a gene for familial situs abnormalities to human chromosome Xq24-q27.1.
Ambiguous abdominal situs, asplenia/polysplenia and severe cardiac malformations characterize heterotaxy in humans. These anomalies result from the inability of the developing embryo to establish normal left-right asymmetry. We have studied an interesting family in which the heterotaxy phenotype segregates as an X-linked recessive trait. In order to map the heterotaxy locus (HTX), we have analysed 39 family members using highly-polymorphic microsatellite markers from the X chromosome. One of these markers, DXS994, shows no recombination with the disease locus in 20 informative meioses. Linkage analysis results in a maximum lod score of 6.37. Current genetic and physical mapping data assign the order of loci in Xq24-q27.1 as cen-DXS1001-(DXS994, HTX)-DXS984-tel. These results establish the first mapping assignment of situs abnormalities in humans.
Child↗
Coccidioides immitis mastitis in a mare.
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Animals↗
Immunization of human HIV-seronegative volunteers with recombinant p17/p24:Ty virus-like particles elicits HIV-1 p24-specific cellular and humoral immune responses.
OBJECTIVE: To evaluate the immune response to HIV-1 p24 generated in vivo by p17/p24:Ty virus-like particles (p17/p24:Ty-VLP) by examining the lymphoproliferative and antibody (Ab) responses to HIV-1 p24, as well as Gag-specific cytotoxic T lymphocytes (CTL), in HIV-seronegative volunteers immunized with hybrid p17/p24:Ty-VLP. DESIGN AND METHODS: Sixteen HIV-seronegative volunteers were immunized with p17/p24:Ty-VLP at two dose levels (100 or 500 micrograms) and monitored for the following 48 weeks for production of anti-p24 and anti-p17 Ab, in vitro lymphoproliferative responses to HIV-1 p24 and p17, and in vitro CTL responses to HIV-1 Gag. RESULTS: Twelve out of the 16 volunteers had significant p24-specific proliferative responses, with volunteers on the higher dose schedule exhibiting earlier proliferative responses than those on the lower dose schedule. Proliferative responses in both volunteer groups were similar in overall magnitude but appeared at different times during the immunization schedule. Anti-p24 Ab were detected in six out of the nine individuals in the lower dose group and in five out of the seven in the higher dose group. There was a good correlation between the presence of p24-specific Ab and the detection of lymphoproliferative responses to the p24 protein in peripheral blood mononuclear cells isolated from the same individuals. Anti-p17 Ab were detected in five volunteers. No Gag-specific CTL responses were detected. CONCLUSION: We conclude that hybrid HIV-1 p17/p24:Ty-VLP are capable of inducing both cellular and humoral immunity to HIV-1 Gag p17 and p24 components and are worthy of further study as a potential HIV immunotherapeutic.
AIDS Vaccines↗
Prophylactic activity of tetracycline against Brugia pahangi infection in jirds (Meriones unguiculatus).
The ability of oral tetracycline to inhibit the development of third-stage infective larvae (L3) of Brugia pahangi to adult worms in jirds was studied using 2 experimental protocols. Jirds treated with 1.4% tetracycline in drinking water for a period beginning 30 days before inoculation of L3 until 30 days post-inoculation (DPI) had 97% reduction in adult worm recovery compared to untreated controls. Jirds that received 1.2% tetracycline in drinking water beginning 1 day before until either 12 or 26 DPI had adult worm recoveries of 11% and < 1%, respectively. Untreated jirds and those given tetracycline beginning at or later than 13 DPI had similar adult worm recovery (27-29%). Prepatent periods were prolonged, and circulating microfilariae were reduced in jirds given tetracycline from 27 to 54 DPI compared to controls. These data indicate that tetracycline administered to jirds in drinking water inhibits B. pahangi development from L3 to adult worms and suggest that this effect occurs during early larval development. Tetracycline administered to infected jirds prior to and continuing through the onset of patency can also affect development of microfilaremia.
Administration, Oral↗
Pentalogy of Cantrell and ectopia cordis, a familial developmental field complex.
The association of sternal fusion defects with various cardiac, diaphragmatic, and anterior body wall defects represents a developmental field complex that includes the Pentalogy of Cantrell and ectopia cordis. No familial cases have been reported previously. We present 3 consecutively born brothers with extensive diaphragmatic defects, 2 who had the Pentalogy of Cantrell. One of the 2 also had ectopia cordis.
Adult↗
Congenital absence of the vas deferens: recurrence in a family.
We report on 2 brothers with congenital absence of the vas deferens discovered in childhood during inguinal hernia repairs. The vas is absent unilaterally in one sib and bilaterally in the other. The unusual presentation of inguinal hernias in these children is discussed as well as mechanisms of inheritance and associated risk of renal anomalies.
Child, Preschool↗