Corynebacterium jeikeium bacteraemia and pulmonary infiltrates in a patient with acute myelogenous leukaemia.

The authors used vancomycin (VCM) to treat Corynebacterium jeikeium bacteraemia and pulmonary infiltrates in a patient with acute myelogenous leukaemia. Pulmonary infiltrates are found in about one-third of patients with haematological pathologies accompanying C jeikeium bacteraemia. Although linezolid and daptomycin are antimicrobial alternatives for treating Corynebacterium infections, the authors believe that VCM is the best choice for immediate treatment success and to prevent pulmonary infiltrates from developing in the presence of bloodstream infection with Corynebacteria in patients with haematological malignancies.

Diffusion tensor imaging and magnetic resonance spectroscopy of transient cerebral white matter lesions in X-linked Charcot-Marie-Tooth disease.

Background: X-linked Charcot-Marie-Tooth disease (CMTX) is a common inherited axonal or mixed axonal-demyelinating neuropathy. The disease is caused by mutations in the GJB1 gene encoding the gap junction protein connexin32, which is expressed in both Schwann cells in the peripheral nervous system and oligodendrocytes in the central nervous system. Several reports have described CMTX patients presenting with transient and recurrent central nervous system (CNS) symptoms with transient white matter lesions in the brain.

Cell surface expression and internalization of the murine erythroid AE1 anion exchanger tagged with an extracellular FLAG epitope.

Anion exchanger 1 (AE1) is the most abundant integral membrane protein in red cells and is essential for maintaining red cell mechanical stability. However, the mechanism for the assembly of AE1 into the membrane skeletal network remains unknown. Several mutants of murine AE1 tagged with an N-terminal enhanced green fluorescent protein (EGFP) and/or an extracellular FLAG epitope inserted adjacent to the N-glycosylation site were prepared, and their expression was analyzed in HEK293 or COS-1 cells by immunofluorescence microscopy, biotinylation, and deglycosylation.

A Novel Compound Heterozygous Mutation in the CYP4V2 Gene in a Japanese Patient with Bietti's Crystalline Corneoretinal Dystrophy.

Purpose: To describe the clinical and genetic characteristics of a Japanese family in which one member exhibited Bietti's crystalline corneoretinal dystrophy (BCD).

Methods: Using direct sequencing, mutation screening was performed in the CYP4V2 gene of both the patient with BCD and her daughter. Ophthalmic examinations were performed to determine the clinical features of both subjects.

Clathrin-mediated endocytosis of mammalian erythroid AE1 anion exchanger facilitated by a YXXΦ or a noncanonical YXXXΦ motif in the N-terminal stretch.

To explore the roles of the conserved YXXΦ-type motif in the erythroid-specific N-terminal stretch of anion exchanger 1 (AE1), cell surface expression and internalization of various mutants derived from murine erythroid AE1 tagged with an N-terminal enhanced green fluorescent protein and an extracellular FLAG (EGFP-mAE1Flag) were explored in K562 and HEK293 cells. EGFP-mAE1Flag showed rapid internalization, in association with the internalizations of transferrin and the endogenous AE1 chaperone-like protein glycophorin A in K562 cells. Disruption of the conserved Y72VEL sequence markedly reduced the internalization and increased the relative abundance of cell-surface AE1, whereas substitution of the N-terminal region from bovine AE1 that lacks the relevant motif for the corresponding region had less of an effect on internalization.

Correlation of cerebral spinal fluid pH and HCO3- with disease progression in ALS.

Amyotrophic lateral sclerosis (ALS) is a progressive and fatal disease, which is characterized by progressive degeneration of spinal and bulbar innervating motor neurons. However, the underlying mechanisms of motor neuron death remain poorly understood. Several candidate disease biomarkers have been detected in cerebrospinal fluid of ALS patients.

Diffuse large B-cell lymphoma complicated with autoimmune thrombocytopenia.

A patient was diagnosed with autoimmune thrombocytopenia and concurrent diffuse large B-cell lymphoma (DLBCL). Both autoimmune thrombocytopenia and DLBCL were successfully treated using chemotherapy, while steroid therapy, rituximab monotherapy, and high-dose parenteral glucocorticoid therapy combined with high-dose intravenous immunoglobulin failed to improve thrombocytopenia. A greater understanding of the pathogenesis and treatment options can only be definitively clarified by analyses of settings such as the present case, as autoimmune thrombocytopenia is uncommon in patients with malignant lymphoma and the etiology and optimal treatment remain unclear.

Ruptured anterior communicating artery aneurysm associated with anterior cranial fossa dural arteriovenous fistula--case report.

A 77-year-old man presented with an extremely rare association of anterior cranial fossa dural arteriovenous fistula (AVF) with anterior communicating artery (ACoA) aneurysm manifesting as consciousness deterioration due to intracerebral hemorrhage in the left frontal lobe and diffuse subarachnoid hemorrhage. Angiography confirmed the association of a dural AVF fed by both ethmoidal arteries and an ACoA aneurysm. Surgery for these two lesions was performed concurrently, and the ACoA aneurysm was found to be responsible for the hemorrhage.

Inhibitory effects of trehalose on fibroblast proliferation and implications for ocular surgery.

Trehalose is a disaccharide which plays an important role in preserving cells from completely dehydrated circumstances. In this study, we investigated effects of trehalose on proliferative activity of fibroblasts and epithelial cells both in vitro and in vivo. As in vitro assessment, normal human dermal fibroblasts and normal human epidermal keratinocytes were cultured in media containing various concentrations of trehalose.

Extrusion of Na,K-ATPase and transferrin receptor with lipid raft-associated proteins in different populations of exosomes during reticulocyte maturation in dogs.

The present study characterizes canine reticulocyte exosomes. Exosomes are small membrane vesicles involved in membrane remodeling that are released from reticulocytes during the final maturation step of red blood cells. The vesicles collected from reticulocyte culture supernatants by differential centrifugation contained major exosomal proteins including heat shock protein cognate 70 (Hsc70) and transferrin receptors (TfR), consistent with the definition of the exosome.

Activation of mitochondrial calpain and release of apoptosis-inducing factor from mitochondria in RCS rat retinal degeneration.

The present study was performed to investigate changes of cytosolic and mitochondrial calpain activities, and effects of intravitreously injected calpain inhibitor on photoreceptor apoptosis in Royal College of Surgeon's (RCS) rats. Time courses of activities for both cytosolic and mitochondrial calpains and amount of calpastatin in RCS rat retina were analyzed by subcellular fractionation, calpain assay and western blotting. Calpain assay was colorimetrically performed using Suc-LLVY-Glo as substrate.

S-opsin protein is incompletely modified during N-glycan processing in Rpe65(-/-) mice.

Retinal pigment epithelium-specific protein 65 kDa (RPE65) is a key enzyme for the visual cycle in the eye. Rpe65(-/-) mice lack 11-cis-retinal, and show early cone degeneration and mislocalization of cone opsins. The present study investigated whether abnormal modification of cone opsins at the protein level is present in Rpe65(-/-) mice.

Parallel reductions in stomatin and Na,K-ATPase through the exosomal pathway during reticulocyte maturation in dogs: stomatin as a genotypic and phenotypic marker of high K(+) and low K(+) red cells.

Dogs can be divided into two genetic groups (a minor HK phenotype and a major LK phenotype) based on erythrocyte monovalent cation concentrations, which are controlled by the putative hk and lk allelic genes. HK dogs retain Na,K-ATPase in their erythrocytes due to the high activity of the enzyme in their precursor cells, whereas total loss of reticulocyte Na,K-ATPase occurs in LK dogs. Here, we report that the levels of the lipid raft-associated membrane protein stomatin decrease in parallel with those of Na,K-ATPase during reticulocyte maturation due to its extrusion in exosomes.

The covalent modification of spectrin in red cell membranes by the lipid peroxidation product 4-hydroxy-2-nonenal.

Spectrin strengthens the red cell membrane through its direct association with membrane lipids and through protein-protein interactions. Spectrin loss reduces the membrane stability and results in various types of hereditary spherocytosis. However, less is known about acquired spectrin damage.

Structural implications of the EL(KIQ)(L/C)LD(A/G)DD sequence in the C-terminal cytoplasmic tail for proper targeting of anion exchanger 1 to the plasma membrane.

While the C-terminal cytoplasmic tail of anion exchanger 1 (AE1, band 3) has been reported to possess important physiological roles, including one for proper membrane trafficking, its precise characteristics remain unclear. To clarify the overall structural consequences of the conserved sequence EL(K/Q)(L/C)LD(A/G)DD, containing the core binding sequence LDADD for carbonic anhydrase II, in the C-terminal region, we analyzed the membrane expression and turnover of bovine AE1 with a series of truncation and substitution mutations in HEK293 cells. Immunofluorescence microscopy and cell-surface biotinylation demonstrated that truncation mutants missing 18 C-terminal residues targeted the plasma membrane, but the one lacking the conserved region, by truncation of 28 amino acid residues, was retained inside the cells.

Crystal deposits on the lens capsules in Bietti crystalline corneoretinal dystrophy associated with a mutation in the CYP4V2 gene.

Purpose: We report a patient (Case 1) with Bietti crystalline corneoretinal dystrophy (BCD) associated with previously unknown findings of crystal-like deposits on the anterior and posterior lens capsules. This patient is one of four (Cases 1-4) in whom we have found BCD associated with the same mutation in the CYP4V2 gene.

Methods: We present a case report with molecular diagnosis.

Identification of genes for two major sialoglycoproteins, glycophorin A and glycophorin C in canine red cell membranes.

Glycophorins are the major sialoglycoproteins in red blood cell membranes, possessing various physiological and pathological roles. We examined membrane glycoproteins in canine red cells and cloned cDNAs for two major glycophorins, glycophorins A (GPA) and C (GPC) from bone marrow cells. Periodic acid-Schiff staining and immunoblotting analyses showed that canine red cell membranes contained several glycoproteins immunoreactive to an anti-bovine GPC antibody, whereas the most abundant sialoglycoproteins, the candidates for GPA, did not react with an anti-human GPA antibody.

DC-HIL is a negative regulator of T lymphocyte activation.

T-cell activation is the net product of competing positive and negative signals transduced by regulatory molecules on antigen-presenting cells (APCs) binding to corresponding ligands on T cells. Having previously identified DC-HIL as a receptor expressed by APCs that contains an extracellular immunoglobulin (Ig)-like domain, we postulated that it plays a role in T-cell activation. To probe this function, we created soluble recombinant DC-HIL, which we observed to bind activated (but not resting) T cells, indicating that expression of the putative ligand on T cells is induced by activation.

Dectin-2 is a pattern recognition receptor for fungi that couples with the Fc receptor gamma chain to induce innate immune responses.

Antigen presenting cells recognize pathogens via pattern recognition receptors (PRR), which upon ligation transduce intracellular signals that can induce innate immune responses. Because some C-type lectin-like receptors (e.g.

Changes in extracellular neurotransmitters in the cerebrum of familial idiopathic epileptic shetland sheepdogs using an intracerebral microdialysis technique and immunohistochemical study for glutamate metabolism.

Intracerebral microdialysis combined with electroencephalographic recordings was performed on 4 dogs of a familial idiopathic epileptic Shetland sheepdog colony to identify the kinds of neurotransmitters responsible for seizure activity. Immunohistochemistry using glutamate (Glu), glutamate transporter (GLT-1 and GLAST), and glutamine synthetase (GS) antibodies was also carried out on the cerebrum of four familial dogs that died of status epilepticus (SE). High values for extracellular levels of Glu and aspartate (ASP) were detected in association with an increased number of spikes and sharp waves during hyperventilation in 3 of 4 the familial epileptic dogs.

Selective expression of vacuolar H+-ATPase subunit d2 by particular subsets of dendritic cells among leukocytes.

Dendritic cells (DC) are far more potent to activate T cells than other antigen presenting cells (e.g., macrophages) and distributed to many organs where DC develop to functionally and phenotypically distinctive subsets.