T-cell Large Granular Lymphocytic Leukemia with a STAT3 mutation successfully treated with Cord Blood Transplantation.

A 64-year-old woman presented with agranulocytosis, anemia, and bacteremia, leading to a diagnosis of T-cell large granular lymphocytic leukemia (T-LGLL). A molecular analysis identified a signal transducer and activator of transcription 3 (STAT3) Y640F variant. Initial treatment with cyclophosphamide and prednisolone did not improve her condition, but serious infections were observed.

Diffuse Large B-cell Lymphoma Complicated with Anti-3-hydroxy-3-methylglutaryl-Coenzyme A Reductase Immune-mediated Necrotizing Myopathy: A Case Report.

A 75-year-old woman presented with significant muscle weakness after statin use. A muscle biopsy revealed necrotizing myopathy, and the patient tested positive for serum anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies, leading to a diagnosis of anti-HMGCR immune-mediated necrotizing myopathy (IMNM). Computed tomography revealed intraperitoneal lymphadenopathy, which was diagnosed as a diffuse large B-cell lymphoma.

Metabolic state switches between morning and evening in association with circadian clock in people without diabetes.

Aims/introduction: Understanding morning-evening variation in metabolic state is critical for managing metabolic disorders. We aimed to characterize this variation from the viewpoints of insulin secretion and insulin sensitivity, including their relevance to the circadian rhythm.

Materials And Methods: A total of 14 and 10 people without diabetes were enrolled, and underwent a 75-g oral glucose tolerance test (OGTT) and hyperinsulinemic-euglycemic clamp study, respectively.

Clock Gene Dysregulation Induced by Chronic ER Stress Disrupts β-cell Function.

In Wfs1A/a islets, in association with endoplasmic reticulum (ER) stress, D-site-binding protein (Dbp) expression decreased and Nuclear Factor IL-3 (Nfil3)/E4 Promoter-binding protein 4 (E4bp4) expression increased, leading to reduced DBP transcriptional activity. Similar alterations were observed with chemically-induced ER stress. Transgenic mice expressing E4BP4 under the control of the mouse insulin I gene promoter (MIP), in which E4BP4 in β-cells is expected to compete with DBP for D-box, displayed remarkable glucose intolerance with severely impaired insulin secretion.

[Wolfram syndrome: clinical features, molecular genetics of WFS1 gene].

Wolfram syndrome(WFS: OMIM 222300) is a rare recessive neuro-endocrine degenerative disorder, known as DIDMOAD(Diabetes Insipidus, early-onset Diabetes Mellitus, Optic Atrophy and Deafness) syndrome. Most affected individuals carry recessive mutations in the Wolfram syndrome 1 gene(WFS1). The WFS1 protein is an endoplasmic reticulum(ER) embedded protein, which functions in ER calcium homeostasis and unfolded protein responses.

X-box binding protein 1 is essential for insulin regulation of pancreatic α-cell function.

Patients with type 2 diabetes (T2D) often exhibit hyperglucagonemia despite hyperglycemia, implicating defective α-cell function. Although endoplasmic reticulum (ER) stress has been suggested to underlie β-cell dysfunction in T2D, its role in α-cell biology remains unclear. X-box binding protein 1 (XBP1) is a transcription factor that plays a crucial role in the unfolded protein response (UPR), and its deficiency in β-cells has been reported to impair insulin secretion, leading to glucose intolerance.

Fe and P solubilization under limiting conditions by bacteria isolated from Carex kobomugi roots at the Hasaki coast.

Our objective was simply to report a sedge species, Carex kobomugi Ohwi that has beneficial bacterial associations under low Fe and P conditions of the Hasaki coast, Japan. C. kobomugi is the dominant species in our study area and grows closest to the sea.

Progressive multifocal leukoencephalopathy in a patient with multiple myeloma.

Progressive multifocal leukoencephalopathy (PML) is a neurological disease that affects immunodeficient patients. We describe here a case of 64-year-old man with IgD type multiple myeloma (MM) who developed progressive neurological symptoms. T(2)-weighted magnetic resonance imaging of the brain showed a hyperintense non-enhancing lesion in the left frontal lobe, and analysis of the cerebrospinal fluid by polymerase chain reaction revealed the presence of John Cunningham virus (JCV) DNA.

Systemic AL amyloidosis with disseminated intravascular coagulation associated with hyperfibrinolysis.

Many coagulation abnormalities are known to coexist in patients with AL amyloidosis; however, disseminated intravascular coagulation (DIC) is rarely observed. We describe the case of a 61-year-old woman who presented with systemic purpura, macroscopic hematuria, and hepatosplenomegaly as the initial manifestations of systemic AL amyloidosis. A coagulation study revealed severe DIC associated with fibrinolysis.

The antifungal compound totarol of Thujopsis dolabrata var. hondai seeds selects for fungi on seedling root surfaces.

Hinoki-asunaro (Thujopsis dolabrata Sieb. et Zucc. var.

Endoplasmic reticulum stress induces Wfs1 gene expression in pancreatic beta-cells via transcriptional activation.

Objective: The WFS1 gene encodes an endoplasmic reticulum (ER) membrane-embedded protein. Homozygous WFS1 gene mutations cause Wolfram syndrome, characterized by insulin-deficient diabetes mellitus and optic atropy. Pancreatic beta-cells are selectively lost from the patient's islets.