The reactivities and ionization properties of the active-site dithiol groups of mammalian protein disulphide-isomerase.

1. The number of reactive thiol groups in mammalian liver protein disulphide-isomerase (PDI) in various conditions was investigated by alkylation with iodo[14C]acetate. 2.

Visual attention modulates signal detectability.

The mechanism by which visual-spatial attention affects the detection of faint signals has been the subject of considerable debate. It is well known that spatial cuing speeds signal detection. This may imply that attentional cuing modulates the processing of sensory information during detection or, alternatively, that cuing acts to create decision bias favoring input at the cued location.

Implantable sensor for intraoperative and postoperative monitoring of blood flow: a preliminary report.

We have developed an implantable 20 MHz Doppler flow sensor, to monitor blood flow in patients after operation. During surgery the sensor is wrapped around a blood vessel and secured in place with a releasable tie. No tissue puncturing techniques are required.

Nasopharyngeal carcinoma in children--a retrospective review and demonstration of Epstein-Barr viral genomes in tumor cell cytoplasm: a report of the Pediatric Oncology Group.

Twenty-seven nasopharyngeal carcinomas were entered in the Pediatric Oncology Group Rare Tumor Registry from 1973 to 1988 (15 males, 12 females; 10 white, 15 black, two unknown; aged 8 to 17 years). Eight tumors were non-keratinizing carcinomas (World Health Organization 2) and 19 were undifferentiated (World Health Organization 3). The overall 3-year survival rate was 70% (SE 11%).

The diagnostic utility of desmin. A study of 584 cases and review of the literature.

The diagnostic utility of several antibodies against desmin and their optimal staining conditions have not been systematically evaluated. Sections of paraffin-embedded tissues from 584 cases were stained with a monoclonal antibody against desmin (Clone DER 11 from DAKO), using an avidin-biotin-peroxidase technique. The results were tabulated and compared with those from previous reports.

Elevated synthesis of human alpha 1-antitrypsin hinders the secretion of murine alpha 1-antitrypsin from hepatocytes of transgenic mice.

alpha 1-Antitrypsin (AAT) is a major hepatic secretory protein. The elevated synthesis of human AAT within hepatocytes of transgenic mice results in its accumulation within a subset of distended cisternae of the rough endoplasmic reticulum. The protein does not accumulate in large insoluble aggregates as is the case for the human PiZ AAT variant.

Rat liver type I iodothyronine deiodinase is not identical to protein disulfide isomerase.

This study was done to test the recent hypothesis (Boado et al. (1988) Biochem. Biophys.

Adjustment of caloric intake based on self-monitoring in noninsulin-dependent diabetes mellitus: development and feasibility.

It has been recommended that patients who perform self-blood glucose monitoring use the information gained from the test to alter their diabetic regimen. Skyler et al. have proposed algorithms for patients to adjust their insulin dosage when blood sugar is elevated.

Two-dimensional echocardiographic determination of ventricular septal defect size: correlation with autopsy.

The presence of a large ventricular septal defect (VSD) may affect the surgical treatment of congenital heart defects. VSD size and shape by two-dimensional echocardiography (2DE) and at autopsy were compared in 18 patients, average age 6 months, who had 2DE studies shortly prior to death. Six had isolated VSD and the other 12 had additional cardiac anomalies.

Tracheal agenesis. A case report and literature review.

Tracheal agenesis (TA) is a rare congenital anomaly that is incompatible with prolonged life. It occurs in isolation or in association with other anomalies. The affected neonate presents with respiratory distress and is unable to produce an audible cry despite obvious physical effort.

Preparation and characterization of dog pancreas microsomal membranes specifically depleted of protein disulphide-isomerase.

1. The selective release of protein disulphide-isomerase from dog pancreas and rat liver microsomal membranes was studied to throw light on the mechanisms of retention of this enzyme within the endoplasmic reticulum, and in order to prepare microsomal membranes specifically depleted of the enzyme. 2.

Role of protein disulphide-isomerase in the expression of native proteins.

Formation of native disulphide bonds is a post-translational modification associated with the folding and assembly of secretory proteins. The process is catalysed within the lumen of the endoplasmic reticulum by the enzyme protein disulphide-isomerase (PDI), which is abundant in secretory cells and catalyses thiol: protein-disulphide interchange in vitro with very broad protein substrate specificity. The presence of PDI within microsomal vesicles is essential for efficient and rapid cotranslational disulphide bond formation during protein synthesis in vitro.

Comparative study of suture and laser-assisted anastomoses in rat sciatic nerves.

Conventional suture repair of peripheral nerves results in a fibrotic reaction that is detrimental to nerve regeneration. As an alternative procedure known as "laser-assisted" repair, a laser can be used, along with a reduced number of sutures, to reanastomose served peripheral nerves. To explore the long-term implications of this technique, the right sciatic nerves of Sprague-Dawley rats were surgically cut and reanastomosed either by means of four epineurial sutures or two epineurial sutures and CO2 laser welds.

Prenatal diagnosis of dominant and recessive dystrophic epidermolysis bullosa: application and limitations in the use of KF-1 and LH 7:2 monoclonal antibodies and immunofluorescence mapping technique.

Prenatal diagnosis is now possible for junctional and recessive dystrophic forms of epidermolysis bullosa (EB); however, there is no similar published experience for dominant dystrophic EB, although data with KF-1 monoclonal antibody suggests that both forms of dystrophic EB can be identified at least postnatally with this unique probe. We now report our experience with light microscopy, electron microscopy, immunofluorescence mapping, and KF-1 and LH 7:2 monoclonal antibodies, in both a mother with dominant dystrophic EB and her fetus at risk, and in a fetus previously shown to be affected with recessive dystrophic EB. KF-1 and LH 7:2 antigens were absent in recessive dystrophic EB fetal skin, identical to findings observed postnatally.

Multiple tissues express alpha 1-antitrypsin in transgenic mice and man.

Hepatocytes are considered to be the predominant source of alpha 1-antitrypsin (AAT), the major antiprotease in human plasma. The development of emphysema in the hereditary PiZ AAT deficiency state suggests that inhibition of leukocyte elastase in the lung is a major function of this protein. In addition, patients with AAT deficiency are at increased risk for developing cholestasis in infancy and chronic liver disease as adults.

Graft-versus-host disease: gastrointestinal involvement with a rectovaginal fistula.

Bone marrow transplantation is an accepted form of treatment for aplastic anemia and end-stage hematological malignancies. Despite a satisfactory outcome in selected patients, more than one-half develop acute graft-versus-host disease (GVHD), characterized typically by involvement of the skin, liver, and gastrointestinal tract. We describe an unusual case in which a 10-year-old girl developed gastrointestinal symptoms as the only manifestation of GVHD.

Endovascular papillary angioendothelioma in the spleen.

Endovascular papillary angioendothelioma is a rare vascular neoplasm originally described as involving the skin and subcutaneous tissues. No case has been reported to involve the spleen, although structurally similar splenic neoplasms have been reported under different names. A 5-year-old boy presented with isolated splenomegaly and was found to have endovascular papillary angioendothelioma of the spleen.

Anaphylactic shock due to cow's milk protein hypersensitivity in a breast-fed infant.

A newborn infant developed anaphylactic shock three times, once after ingesting his mother's milk and once after ingesting a formula containing casein hydrolysate. Symptoms resolved after cow's milk was withdrawn from the mother's diet and lactation was resumed. The case demonstrates that anaphylactic shock may occur in breast-fed infants and emphasizes the need to consider allergic phenomena even in these infants.

Gallbladder perforation. An imaging analysis.

We evaluated the radiologic studies of 23 patients with surgically or autopsy-documented gallbladder perforation. Extravasation was shown by cholescintigraphy and cholangiography in seven patients and corresponded to free perforation or large pericholecystic loculation. In 16 patients, ultrasonography and computed tomography detected fluid and abscesses outside the gallbladder ranging from 1 to 2 mm pericholecystic fluid collections to large phlegmonous masses.

Rhabdomyosarcoma in children. Correlation of form and prognosis in one institution's experience.

All cases of rhabdomyosarcoma and undifferentiated sarcoma accessioned at Texas Children's Hospital since 1954 were reviewed. Electron microscopy and immunohistochemistry were used as aids to diagnosis. Rhabdomyosarcoma was diagnosed by study of biopsy specimens obtained prior to therapy in 63 of these 105 cases.