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Biomedical subjects

A Kornberg

Publications and source records attributed to A Kornberg.

At least 37 records · Page 2Linked to original sources

Factor VII deficiency in a patient with retinal arteriolar tortuosity syndrome.

PURPOSE: To report a pedigree with hereditary retinal arteriolar tortuosity with macular haemorrhage and abnormality of the coagulation system. METHODS: Case report and literature review. RESULTS: A 49-year-old woman was referred due to macular haemorrhage in both eyes. Her 16-year-old son had recurrent retinal haemorrhages which presented at age 16 years and had mild retinal arteriolar tortuosity. Coagulation studies in the son revealed normal activated partial thromboplastin time (APTT), prolonged prothrombin time (PT) and 30% activity of factor VII. CONCLUSIONS: Factor VII deficiency may aggravate the haemorrhages in retinal arteriolar tortuosity syndrome. We therefore suggest conducting routine coagulation studies (PT, APTT) in all patients with retinal arteriolar tortuosity syndrome. Determination of factor VII activity is warranted only in patients with normal APTT and prolonged PT.

Adolescent↗

Inorganic polyphosphate is required for motility of bacterial pathogens.

The ppk gene encodes polyphosphate kinase (PPK), the principal enzyme in many bacteria responsible for the synthesis of inorganic polyphosphate (polyP) from ATP. A null mutation in the ppk gene of six bacterial pathogens renders them greatly impaired in motility on semisolid agar plates; this defect can be corrected by the introduction of ppk gene in trans. In view of the fact that the motility of pathogens is essential to invade and establish systemic infections in host cells, this impairment in motility suggests a crucial and essential role of PPK or polyP in bacterial pathogenesis.

Bacteria↗

Inorganic polyphosphate in Vibrio cholerae: genetic, biochemical, and physiologic features.

Vibrio cholerae O1, biotype El Tor, accumulates inorganic polyphosphate (poly P) principally as large clusters of granules. Poly P kinase (PPK), the enzyme that synthesizes poly P from ATP, is encoded by the ppk gene, which has been cloned from V. cholerae, overexpressed, and knocked out by insertion-deletion mutagenesis. The predicted amino acid sequence of PPK is 701 residues (81.6 kDa), with 64% identity to that of Escherichia coli, which it resembles biochemically. As in E. coli, ppk is part of an operon with ppx, the gene that encodes exopolyphosphatase (PPX). However, unlike in E. coli, PPX activity was not detected in cell extracts of wild-type V. cholerae. The ppk null mutant of V. cholerae has diminished adaptation to high concentrations of calcium in the medium as well as motility and abiotic surface attachment.

Amino Acid Sequence↗

Differentiated therapy with prostaglandin E1 (alprostadil) after orthotopic liver transplantation: the usefulness of procalcitonin (PCT) and hepatic artery resistive index (RI) for the evaluation of early graft function and clinical course.

Increasing demand for donor organs has led to new pharmacological concepts for reducing ischemia-reperfusion injury (I/R) of the graft after liver transplantation to prevent primary non-functioning of the organ. Prostaglandins have proved to be cytoprotective in several experimental models of ischemia and transplantation. The prophylactic administration after orthotopic liver transplantation is still a subject of controversial discussion. The aim of our study was the evaluation of the post-transplant hepatic artery resistive index (RI) measured by color Doppler imaging, in combination with postoperative elevation of transaminases, as parameters indicating the need for a differentiated systemic therapy with prostaglandin E1 (PGE1) (alprostadil). In addition, the value of serum procalcitonin (PCT) as a postoperative parameter for the extent of I/R is investigated. In the case of post-transplant elevated hepatic artery RI (RI > 0.75), the administration of PGE1 led to a significant reduction of transaminases (p < 0.05) and a decline of the RI. In addition, postoperative PCT levels could be reduced significantly by PGE1 application. These results suggest that determination of RI is feasible for indicating a need for therapy with PGE1. Its targeted application reduces hepatocellular damage due to I/R after liver transplantation.

Alprostadil↗

Impact of Ig-Therasorb immunoapheresis on stability of xenogeneic ex vivo porcine liver perfusion--value of aminotransferases and flow rates for the assessment of metabolic graft viability.

Due to growing shortage of donor organs, the concept of extracorporeal pig liver perfusion in the treatment of acute liver failure has been rediscovered. Immunomodulation, such as immunoapheresis or inhibition of complement, results in long-term perfusion without exact knowledge of the remaining metabolic graft viability. This study was aimed at the comparison of conventional parameters of graft stability such as perfusion rates and release of aminotransferases with parameters of metabolic graft function. Ig-Therasorb immunoapheresis (IA) of the xenogeneic perfusate was performed to protect the discordant pig livers from hyperacute rejection, mediated by preformed naturally occurring human xenogeneic antibodies. The application of IA created stable autologous graft reperfusion after a short time of xenoperfusion, but it was not able to prevent the livers from severe synthetic and functional damage. In the future, improvement of xenogeneic graft function, rather than pure prolongation of perfusion, must be the principal aim.

Animals↗

Anti-beta 2-glycoprotein I antibodies and anti-endothelial cell antibodies induce tissue factor in endothelial cells.

Anti-beta 2-glycoprotein I antibodies bind to endothelial cells through beta 2-GPI. The antibodies are present in patients with systemic lupus erythematosus and antiphospholipid syndrome and are associated with the pathogenesis of the disease. Anti-endothelial cell antibodies that react with constitutive antigens on ECs are present in patients with vasculiditis and other diseases. Both types of antibodies can activate ECs. Frequent findings in APLS and vasculitis are fibrin deposits and thromboembolic phenomena. These indicate that the coagulation system is activated. However, the mechanism of activation is not clear. ECs generate tissue factor upon stimulation with various substances. In the present study we report that monoclonal anti-beta 2-GPI antibodies and AECAs, derived from a patient with primary APLS and a patient with Takayasu's arteritis, respectively, induce a potent tissue factor in ECs. The production of TF activity, TF antigen and TF mRNA is dose and time dependent. The TF activity was induced also by F(ab)2 but not by Fc fragments and was abolished completely by pre-incubation with ant-TF antibodies. The TF that is induced in ECs by AECAs with and without beta 2-GPI specificity may activate the coagulation and thereby play a major role in the pathogenesis of fibrin deposition and thrombus formation in diseases that are associated with the presence of these antibodies.

Antibodies↗

Inducible nitric oxide synthase activation after ischemia/reperfusion contributes to myocardial dysfunction and extent of infarct size in rabbits: evidence for a late phase of nitric oxide-mediated reperfusion injury.

BACKGROUND: Ischemia/reperfusion (I/R) leads to the induction of inducible nitric oxide synthase. The present study investigated the effects of selective and continuous inhibition of iNOS on myocardial performance, infarct size and histomorphological changes after I/R in rabbits. METHODS AND RESULTS: Ischemia/reperfusion (I/R) was induced by occlusion of the circumflex coronary artery for 30 min followed by 48 h of reperfusion. Sham animals (group A) served as control. Three groups were subjected to I/R: (B) placebo; (C) aminoguanidine (AMG; 10 mg/kg bolus) given prior to and 48 h after I/R to test its acute effects; (D) AMG (300 mg/kg/day s.c.) to test effects of continuous treatment. Hemodynamics, myocardial blood flow, infarct size, iNOS activity, cGMP levels, immunohistochemical analysis of iNOS expression and AMG tissue levels were determined. Continuous AMG treatment improved myocardial performance (hemodynamics and blood flow) compared to placebo group. iNOS was highest in placebo-treated animals. AMG tissue levels were highest in tissues affected by I/R. Infarct size (% of the circumflex region) was significantly smaller in group D when compared to group B. CONCLUSIONS: This is the first study showing that activation of myocardial iNOS isozyme during 48 h of reperfusion contributes to a late phase of I/R-induced injury in rabbits. Selective and continuous modulation of iNOS by AMG over this time period exerts protective effects with respect to myocardial performance, coronary blood flow, cellular infiltration and reduction of infarct size; this may be a novel therapeutic approach in the clinical situation to limit irreversible myocardial injury associated with ischemia and reperfusion.

Adrenergic beta-Agonists↗

Inorganic polyphosphate: a molecule of many functions.

Pursuit of the enzymes that make and degrade polyP has provided analytic reagents which confirm the ubiquity of polyP in microbes and animals and provide reliable means for measuring very low concentrations. Many distinctive functions appear likely for polyP depending on its abundance, chain length, biologic source and subcellular location: an energy supply and ATP substitute, a reservoir for Pi, a chelator of metals, a buffer against alkali, a channel for DNA entry, a cell capsule, and, of major interest, a regulator of responses to stresses and adjustments for survival in the stationary phase of culture growth and development. Whether microbe or human, we depend on adaptations in the stationary phase, a dynamic phase of life. Much attention has focused on the early and reproductive phases of organisms, rather brief intervals of rapid growth, but more concern needs to be given to the extensive period of maturity. Survival of microbial species depends on being able to manage in the stationary phase. In view of the universality and complexity of basic biochemical mechanisms, it would be surprising if some of the variety of polyP functions observed in microorganisms did not apply to aspects of human growth and development, to aging and to the aberrations of disease. Of theoretical interest regarding polyP is its antiquity in prebiotic evolution, which, along with its high energy and phosphate content, make it a plausible precursor to RNA, DNA and proteins. Of practical interest is its many industrial applications, among which is its use in the microbial depollution of Pi in marine environments.

Amino Acid Sequence↗

Inorganic polyphosphate regulates responses of Escherichia coli to nutritional stringencies, environmental stresses and survival in the stationary phase.

The molecular mechanisms responsible for polyP accumulation in E. coli remain largely obscure. Based on the available data, a tentative model is proposed (Fig. 1; Ault-Riché et al. 1998). Inhibition by (p)ppGpp of PPX interrupts the dynamic balance between the synthesis of polyP by PPK and its hydrolysis by PPX, accounting for polyP accumulation. However, mutants lacking PhoB, the response regulator of the Pho regulon, fail to accumulate polyP even in the face of high levels of (p)ppGpp. Clearly, PhoB is required in some undefined manner. With regard to osmotic stress, the pathway to polyP accumulation is also distinct from the one identified with the activation of envZ and the associated changes in membrane functions. A tentative scheme attempting to describe the metabolic turnover of polyP is given in Fig. 4. [figure: see text] In adaptations to stress, cells must coordinate major changes in the rates of transcription, translation, and replication as well as make choices in the genes expressed (Kolter et al. 1993). PolyP could provide activated phosphates or coordinate an adaptive response by binding metals and/or specific proteins. Accumulation of polyP in E. coli and other organisms is commonly assumed to provide a reservoir of energy convertible to ATP. This seems implausible because of the turnover of ATP which consumes only a fraction of a second (Chapman and Atkinson 1977). Thus, other functions for polyP need to be considered, among them a regulatory role. PolyP, even at very low levels, is essential in E. coli for adaptations in stationary phase and for survival (Rao and Kornberg 1996). As a polyanionic polymer, polyP has chemical similarities to DNA and RNA in interactions with basic domains of proteins. Further investigation of the cellular location of polyP, its state of metabolic availability and identification of its binding partners are needed. In view of the ubiquity of polyP in eukaryotic cells (including dynamic turnover in the nuclei of some mammalian cells), studies similar to those undertaken in E. coli may reveal comparable functions.

Adaptation, Physiological↗

Leaflet escape in Omnicarbon monoleaflet valve.

In almost every type of artificial valve, mechanical disruption has been described. We present the first case of leaflet fracture with aortic embolization in an Omnicarbon monoleaflet valve 42 months after implantation. The 22-year-old male patient suffered of acute respiratory insufficiency and was referred to our hospital as an emergency case. Until a few days before presentation, he was in good condition without clinical complaints. By means of transesophageal echocardiography (TEE) and abdominal sonography the diagnosis of leaflet fracture with embolization to the abdominal aorta could be made. The patient underwent consecutive operative valve replacement and foreign body extraction that resulted in a complete recovery.

Adult↗

Impact of immunoadsorption on xenogeneic extracorporeal pig liver perfusion: assessment of organ function during autologous reperfusion.

The shortage of liver grafts has resulted in an intensive search for alternative strategies of liver support in acute liver failure. Extracorporeal perfusion of pig livers (ECLP) is not a new concept, but recent improvements in cardiopulmonary bypass technology and new knowledge about xenogeneic immunologic mechanisms have made it a potential therapeutic modality. In previous suboptimal experimental and clinical models, no statements about the beneficial effect of long-lasting periods of ECLP were made. The aim of this study was to evaluate the capacity of pig liver for metabolic regeneration after xenogeneic ECLP under largely physiologic conditions. To delay hyperacute rejection (HAR), we utilized immunoadsorption (IA) of naturally preformed xenogeneic antibodies (XNAbs). This led to a stable xenogeneic ECLP for 45 min. However, IA was not able to prevent completely organ damage or loss of function resulting from insufficient autologous reperfusion. The inability to regenerate during autologous reperfusion provides a new measure of the function of a xenogeneic perfused pig liver. In addition, our results implicate that patients with fulminant liver failure will benefit from short perfusions with several fresh organs rather than from long perfusion with a single pig liver.

Animals↗

Aminoguanidine inhibits inducible NOS and reverses cardiac dysfunction late after ischemia and reperfusion--implications for iNOS-mediated myocardial stunning.

BACKGROUND: The functional significance of inducible nitric oxide synthase (iNOS) activation in response to myocardial ischemia and reperfusion (I/R) was investigated. METHODS: New Zealand rabbits were randomly treated with either placebo, aminoguanidine (AMG; selective iNOS inhibitor), or L-arginine. Left-ventricular hemodynamics and myocardial blood flow were measured before coronary occlusion and 30 minutes and 48 h after initiation of reperfusion. RESULTS: I/R resulted in left-ventricular dysfunction and increased myocardial iNOS activity. Placebo treatment had no effects on myocardial function. However, AMG significantly inhibited iNOS activity, significantly improved left-ventricular maximum + dP/dt and decreased LVEDP, whereas administration of L-arginine reduced + dP/dt and slightly increased LVEDP, compared to AMG-treated animals. Myocardial blood flow in the affected myocardium significantly increased after both AMG and L-arginine. CONCLUSIONS: The present data indicate that induction of myocardial iNOS after 48 h I/R contributes to the development of reversible left-ventricular dysfunction, suggesting the involvement of iNOS in myocardial stunning. Whereas L-arginine is associated with further reduction of left-ventricular contractility, continuous inhibition of iNOS activation by AMG improves left-ventricular performance; this may be a novel and clinically important therapeutic modality in certain disease states associated with I/R, including cardiac operations using extracorporeal circulation and coronary angioplastic procedures.

Animals↗

Inorganic polyphosphate: a molecule of many functions.

Inorganic polyphosphate (poly P) is a chain of tens or many hundreds of phosphate (Pi) residues linked by high-energy phosphoanhydride bonds. Despite inorganic polyphosphate's ubiquity--found in every cell in nature and likely conserved from prebiotic times--this polymer has been given scant attention. Among the reasons for this neglect of poly P have been the lack of sensitive, definitive, and facile analytical methods to assess its concentration in biological sources and the consequent lack of demonstrably important physiological functions. This review focuses on recent advances made possible by the introduction of novel, enzymatically based assays. The isolation and ready availability of Escherichia coli polyphosphate kinase (PPK) that can convert poly P and ADP to ATP and of a yeast exopolyphosphatase that can hydrolyze poly P to Pi, provide highly specific, sensitive, and facile assays adaptable to a high-throughput format. Beyond the reagents afforded by the use of these enzymes, their genes, when identified, mutated, and overexpressed, have offered insights into the physiological functions of poly P. Most notably, studies in E. coli reveal large accumulations of poly P in cellular responses to deficiencies in an amino acid, Pi, or nitrogen or to the stresses of a nutrient downshift or high salt. The ppk mutant, lacking PPK and thus severely deficient in poly P, also fails to express RpoS (a sigma factor for RNA polymerase), the regulatory protein that governs > or = 50 genes responsible for stationary-phase adaptations to resist starvation, heat and oxidant stresses, UV irradiation, etc. Most dramatically, ppk mutants die after only a few days in stationary phase. The high degree of homology of the PPK sequence in many bacteria, including some of the major pathogenic species (e.g. Mycobacterium tuberculosis, Neisseria meningitidis, Helicobacter pylori, Vibrio cholerae, Salmonella typhimurium, Shigella flexneri, Pseudomonas aeruginosa, Bordetella pertussis, and Yersinia pestis), has prompted the knockout of their ppk gene to determine the dependence of virulence on poly P and the potential of PPK as a target for antimicrobial drugs. In yeast and mammalian cells, exo- and endopolyphosphatases have been identified and isolated, but little is known about the synthesis of poly P or its physiologic functions. Whether microbe or human, all species depend on adaptations in the stationary phase, which is truly a dynamic phase of life. Most research is focused on the early and reproductive phases of organisms, which are rather brief intervals of rapid growth. More attention needs to be given to the extensive period of maturity. Survival of microbial species depends on being able to manage in the stationary phase. In view of the universality and complexity of basic biochemical mechanisms, it would be surprising if some of the variety of poly P functions observed in microorganisms did not apply to aspects of human growth and development, to aging, and to the aberrations of disease. Of theoretical interest regarding poly P is its antiquity in prebiotic evolution, which along with its high energy and phosphate content, make it a plausible precursor to RNA, DNA, and proteins. Practical interest in poly P includes many industrial applications, among which is the microbial removal of Pi in aquatic environments.

Humans↗

Continuous transesophageal echocardiographic (TEE) monitoring during port-access cardiac surgery.

BACKGROUND: Since the introduction of the closed-chest minimally invasive heart surgery using the Port-Access system a variety of monitoring techniques including fluoroscopy, transesophageal echocardiography (TEE) and invasive pressure measurements have been described. We investigated whether or not single TEE is feasible for perioperative monitoring of the placement, localization and proper function of the endovascular cardiopulmonary bypass (CPB) devices. METHODS: Fifty-one patients (35 mitral valve repair or replacement [MVR], 8 coronary artery bypass grafting [CABG], 5 atrial septal defects [ASD] and 3 left atrial myxoma) were subjected to Port-Access surgery (PAS). Intraoperative Omniplane-TEE (2D- and color-flow Doppler techniques) was used as the leading monitoring device for correct positioning of the endopulmonary vent catheter and the venous cannula, and for the visualization of the guide wire and the endoaortic occlusion catheter (Endoclamp). After balloon inflation, its proper positioning and function during endo-aortic occlusion, sufficient delivery of cardioplegia into the coronary ostia, absence of leakage flow and adequate venting were controlled. Left and right radial artery catheters as well as aortic root pressure measurements served as controls. Additional fluoroscopy was used as standby device. RESULTS: In 46 patients (90.1%) sufficient perioperative monitoring was provided by single TEE. In five cases additional intermittent fluoroscopy was necessary for correct positioning of the guide wire (CABG) and the Endoclamp (three MVR and one ASD). Dislocation of the Endoclamp into the left ventricle was observed once but was successfully corrected by TEE guidance. Weaning from CPB and de-airing were easily guided with TEE. We did not observe balloon-mediated aortic injury or aortic valve dysfunction, and myocardial recovery from CPB was uneventful. All cases of MVRs showed sufficient results (68% without evidence of regurgitation, 32% showed residual mitral valve incompetence of less than grade II). Neither perivalvular leakage (MV-replacement) nor shunt- (residual ASD) flow were detectable. CONCLUSIONS: We recommend single TEE as a safe and effective on-line imaging device for monitoring the endovascular CPB system during PAS. Fluoroscopy with its potential risk for the patients and the staff due to x-ray exposure should only be used in the presence of peripheral vascular disease or when echocardiographic imaging is insufficient.

Cardiopulmonary Bypass↗

Asymptomatic right atrial leiomyosarcoma with tricuspid valve obstruction in a young female patient.

Primary cardiac leiomyosarcomas of the heart are rare tumors usually diagnosed post-mortem. Like other sarcomas located in the heart, it causes symptoms by obstruction or occlusion of cardiac cavities, local invasion, embolization or by systemic manifestations. We present an unusual case of a previously healthy young female patient who was accidentally diagnosed with a cardiac tumor of unknown origin during routine physical examination in May 1997. Until a few days prior to elective cardiac surgery for diagnostic purposes in June 1997, no clinical symptoms were present. To our surprise, a primary right atrial leiomyosarcoma was found which almost completely occluded the right atrium and destroyed the tricuspid valve. Despite the combination of surgical removal and adjuvant chemotherapy the patient died 3 weeks after the operation due to progressive tumor disease and development of congestive heart failure and lung embolism.

Adult↗

Alginate, inorganic polyphosphate, GTP and ppGpp synthesis co-regulated in Pseudomonas aeruginosa: implications for stationary phase survival and synthesis of RNA/DNA precursors.

The regulatory protein AlgR2 in Pseudomonas aeruginosa positively regulates nucleoside diphosphate kinase (Ndk) and succinyl-CoA synthetase, enzymes critical in nucleoside triphosphate (NTP) formation. AlgR2 positively regulates the production of alginate, GTP, ppGpp and inorganic polyphosphate (poly P). An algR2 mutant with low levels of these metabolites has them restored by introducing and overexpressing either the algR2 or the ndk gene into the algR2 mutant. Thus, Ndk is involved in the formation of these compounds and largely prevents the death of the algR2 mutant, which occurs early in the stationary phase. We demonstrate that the 12 kDa Ndk-pyruvate kinase (Pk) complex, previously shown to generate predominantly GTP instead of all the NTPs, has a low affinity for the deoxynucleoside diphosphates and cannot generate the dNTPs needed for DNA replication and cell division; this complex may thus be involved in regulating the levels of both NTPs and dNTPs that modulate cell division and survival in the stationary phase.

Alginates↗