Proteomic analysis of glutathione transferases from the liver fluke parasite, Fasciola hepatica.

The parasite Fasciola hepatica causes major global disease of livestock, with increasing reports of human infection. Vaccine candidates with varying protection rates have been identified by pre-genomic approaches. As many candidates are part of protein superfamilies, sub-proteomics offers new possibilities to systematically reveal the relative importance of individual family proteins to vaccine formulations within populations.

Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children.

Purpose: This study was undertaken to characterize the contemporary surgical treatment of pediatric renovascular hypertension.

Methods: A retrospective analysis was conducted of the clinical data of 97 consecutive pediatric patients (39 girls, 58 boys), aged from 3 months to 17 years, who underwent operation at the University of Michigan from 1963 to 2006. All but one patient had refractory hypertension not responsive to contemporary medical therapy.

Regulation of c-Ret in the developing kidney is responsive to Pax2 gene dosage.

During kidney development, Pax2 and Pax8 are expressed very early in the mammalian nephric duct and both precede the expression of receptor tyrosine kinase, c-Ret. However, in Pax2-/- mutant mice, expression of c-Ret is lost after embryonic day 10.5.

A functional FERM domain binding motif in neurofascin.

The L1 family of transmembrane cell adhesion receptors are involved in the development of the nervous system and consist of L1, neuron-glial-related cell adhesion molecule and neurofascin. All three receptors have a short cytoplasmic tail which is known to bind to the cytoskeletal associated protein ankyrin. Ezrin is a cytoplasmic binding protein known to link plasma membrane proteins to the cytoskeleton and has been shown to be a binding partner for L1.

Disruption of neurofascin localization reveals early changes preceding demyelination and remyelination in multiple sclerosis.

Saltatory conduction in the nervous system is enabled through the intimate association between the leading edge of the myelin sheath and the axonal membrane to demarcate the node of Ranvier. The 186 kDa neuron specific isoform of the adhesion molecule neurofascin (Nfasc186) is required for the clustering of voltage gated Na+ channels at the node, whilst the 155 kDa glial specific isoform (Nfasc155) is required for the assembly of correct paranodal junctions. In order to understand the relationship between these vital structures and how they are affected in multiple sclerosis we have examined the expression of Nfasc155 and Nfasc186 in areas of inflammation, demyelination and remyelination from post-mortem brains.

Mitochondrial DNA sequence diversity in extant Irish horse populations and in ancient horses.

Equine mitochondrial DNA sequence variation was investigated in three indigenous Irish horse populations (Irish Draught Horse, Kerry Bog Pony and Connemara Pony) and, for context, in 69 other horse populations. There was no evidence of Irish Draught Horse or Connemara Pony sequence clustering, although the majority of Irish Draught Horse sequences (47%) were assigned to haplogroup D. Conversely, 31% of the Kerry Bog Pony sequences were assigned to the rare haplogroup E.

Evidence for biogeographic patterning of mitochondrial DNA sequences in Eastern horse populations.

Equine mitochondrial DNA (mtDNA) phylogeny reconstruction reveals a complex pattern of variation unlike that seen in other large domesticates. It is likely that this pattern reflects a process of multiple and repeated, although not necessarily independent, domestication events. Until now, no clear geographic affiliation of clades has been apparent.

Plasticity demonstrated in the proteome of a parasitic nematode within the intestine of different host strains.

The soluble global proteome of adult nematode Heligmosomoides polygyrus (H. p.) bakeri, a hookworm laboratory model was compared for the first time in the intestines of a slow-responder mouse host strain (C57/BL10) that is known to support a primary parasite infection for many months, and rapid-responder mouse host (SWR) that is known to eliminate the nematode infection by week 6 postinfection.

A novel nonsense mutation in the EYA1 gene associated with branchio-oto-renal/branchiootic syndrome in an Afrikaner kindred.

Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder characterized by the associations of hearing loss, branchial arch defects and renal anomalies. Branchiootic (BO) syndrome is a related disorder that presents without the highly variable characteristic renal anomalies of BOR syndrome. Dominant mutations in the human homologue of the Drosophila eyes absent gene (EYA1) are frequently the cause of both BOR and BO syndromes.

Renal replacement therapy in the treatment of confirmed or suspected inborn errors of metabolism.

Objective: Analysis of mortality and risk factors for mortality in the use of renal replacement therapy to correct metabolic disturbances associated with confirmed or suspected inborn errors of metabolism.

Study Design: A retrospective review of an institutional review board-approved pediatric acute renal failure data base at the University of Michigan. Eighteen patients underwent 21 renal replacement therapy treatments for metabolic disturbances caused by urea cycle defects (n = 14), organic acidemias (n = 5), idiopathic hyperammonemia (n = 1), and Reye syndrome (n = 1).

Medication errors and patient complications with continuous renal replacement therapy.

Continuous renal replacement therapy (CRRT) is commonly used for renal support in the intensive care unit. While the risk of medication errors in the intensive care unit has been described, errors related specifically to CRRT are unknown. The purpose of this study is to characterize medication errors related to CRRT and compare medication errors that occur with manually compounded solutions versus commercially available solutions.

Accuracy of implanted port placement with the use of the electromagnetic CathTrack catheter locator system.

Purpose: To test whether an electromagnetic guidance system such as CathTrack would allow long-term central venous access devices to be reliably placed at a decreased cost and without radiation exposure to patients and staff. The following study was undertaken to verify accuracy of the CathTrack system for catheter placement and to develop guidelines for its use.

Methods: Twenty-nine consecutive patients were prospectively enrolled in the study and taken to the operating room for implantation of a permanent central venous access port.

Charcot-Marie-Tooth type 4F disease caused by S399fsx410 mutation in the PRX gene.

Charcot-Marie-Tooth type 4F disease (CMT4F) is an autosomal recessive neuropathy caused by mutations in the PRX gene. To date, only seven mutations have been identified in the PRX gene. In this study, the authors report a novel S399fsX410 mutation in the PRX gene and its effects at the protein level, which was identified in an 8-year-old patient with early-onset CMT disease.

Developing clinical protocols for nursing practice: improving nephrology care for children and their families.

Improving and maintaining quality patient care through the development and implementation of clinical protocols is an important facet in our health care system. Identifying the need for clinical protocols, developing and maintaining the protocols, and defining the specific role of pediatric nephrology nurses in this process is presented herein. To illustrate the process we have included two examples of current clinical protocols utilized by our division.

Neurofascins are required to establish axonal domains for saltatory conduction.

Voltage-gated sodium channels are concentrated in myelinated nerves at the nodes of Ranvier flanked by paranodal axoglial junctions. Establishment of these essential nodal and paranodal domains is determined by myelin-forming glia, but the mechanisms are not clear. Here, we show that two isoforms of Neurofascin, Nfasc155 in glia and Nfasc186 in neurons, are required for the assembly of these specialized domains.

Crystal structure of a new class of glutathione transferase from the model human hookworm nematode Heligmosomoides polygyrus.

The crystal structure of GST Nu2-2 (HpolGSTN2-2) from the model hookworm nematode Heligmosomoides polygyrus has been solved by the molecular replacement method and refined to a resolution of 1.71 A, providing the first structural data from a class of nematode-specific GSTs. By structural alignment with two Sigma class GSTs, glutathione could be rationally docked into the G-site of the enzyme.

Biochemical characterization and vaccine potential of a heme-binding glutathione transferase from the adult hookworm Ancylostoma caninum.

We report the cloning and expression of Ac-GST-1, a novel glutathione S-transferase from the adult hookworm Ancylostoma caninum, and its possible role in parasite blood feeding and as a vaccine target. The predicted Ac-GST-1 open reading frame contains 207 amino acids (mass, 24 kDa) and exhibited up to 65% amino acid identity with other nematode GSTs. mRNA encoding Ac-GST-1 was detected in adults, eggs, and larval stages, but the protein was detected only in adult hookworm somatic extracts and excretory/secretory products.

A novel 4.1 ezrin radixin moesin (FERM)-containing protein, 'Willin'.

The 4.1 superfamily of proteins contain a 4.1 Ezrin Radixin Moesin (FERM) domain and are described as linking the cytoskeleton with the plasma membrane.

Mechanisms of axon ensheathment and myelin growth.

The evolution of complex nervous systems in vertebrates has been accompanied by, and probably dependent on, the acquisition of the myelin sheath. Although there has been substantial progress in our understanding of the factors that determine glial cell fate, much less is known about the cellular mechanisms that determine how the myelin sheath is extended and stabilized around axons. This review highlights four crucial stages of myelination, namely, the selection of axons and initiation of cell-cell interactions between them and glial cells, the establishment of stable intercellular contact and assembly of the nodes of Ranvier, regulation of myelin thickness and, finally, longitudinal extension of myelin segments in response to the lengthening of axons during postnatal growth.

Standards of nutritional care in pediatric oncology: results from a nationwide survey on the standards of practice in pediatric oncology. A Children's Oncology Group study.

Background: The prevalence of malnutrition in children with cancer ranges between 8% and 60%. Malnutrition is strongly associated with the nature of treatment and increases an individual's risk of infection. Clinical studies have suggested that nutrition intervention may decrease toxicity and improve survival in the oncology population.

Multi-centre evaluation of anticoagulation in patients receiving continuous renal replacement therapy (CRRT).

Background: Heparin (hepACG) and regional citrate anticoagulation (citACG) remain the most commonly reported continuous renal replacement therapy (CRRT) ACG methods employed. No prospective multi-centre published data exist that compare different ACG methods with respect to CRRT filter life span or patient complications.

Methods: A total of 138 patients from seven US centres receiving 18 208 h of CRRT comprising a total of 442 CRRT circuits were utilized to assess filter life span and ACG-related complications in patients receiving CRRT with hepACG, citACG or no ACG (noACG).

Analysing proteomic data.

The rapid growth of proteomics has been made possible by the development of reproducible 2D gels and biological mass spectrometry. However, despite technical improvements 2D gels are still less than perfectly reproducible and gels have to be aligned so spots for identical proteins appear in the same place. Gels can be warped by a variety of techniques to make them concordant.

High mineral and vitamin E intake by pregnant ewes lowers colostral immunoglobulin G absorption by the lamb.

A 2 x 2 factorial arrangement of treatments with 78 mature ewes was used to evaluate the effects of supplementing the pregnant ewe's diet with high levels of minerals and vitamin E on immunoglobulin G (IgG) absorption by the lamb and whether any altered efficacy of IgG absorption was due to the colostrum or to the lamb. The ewes were estrus-synchronized in October and housed in wk 10 of gestation. In the final 7 wk of gestation, a grass silage-based diet, offered ad libitum, was supplemented with 500 g of a 19% CP concentrate, and from 1 wk later until lambing, half the ewes was offered 48 g of a mineral/vitamin supplement containing 6.

Hyperosmolar solutions in continuous renal replacement therapy for hyperosmolar acute renal failure: a preliminary report.

Objective: To demonstrate the efficacy of hyperosmolar dialysis and prefilter replacement fluid solutions for continuous renal replacement therapies in the correction of hyperosmolar disorders in acute renal failure.

Data Source: An Institutional Review Board-approved pediatric acute renal failure database at the University of Michigan C. S.

Echinococcus granulosus antigen B hydrophobic ligand binding properties.

Antigen B (AgB), an immunodominant component of the cestode parasite Echinococcus granulosus, presents homology to and shares apparent structural similarities with helix-rich hydrophobic ligand binding proteins (HLBPs) from other cestodes. In order to investigate the fatty acid binding properties of AgB, two of its subunit components (rAgB8/1 and rAgB8/2) were expressed in Escherichia coli and purified, and the native antigen was purified from the hydatid cyst fluid by affinity chromatography using a monoclonal antibody raised against rAgB8/1. The interaction of the purified native and recombinant proteins with the fluorescent ligands DAUDA, ANS, DACA and 16-AP was investigated.