Cholesterol hydroperoxide formation in red cell membranes and photohemolysis in erythropoietic protoporphyria

3beta-Hydroxy-5alpha-hydroperoxy-Delta(6)-cholestene is produced in protoporphyrin-containing red blood cell ghosts irradiated with approximately 400-nanometer light in the presence of oxygen. Incorporation of this cholesterol photooxidation product into normal red blood cells leads to increased osmotic fragility and eventual hemolysis. These results may be relevant to photohemolysis associated with erythropoietic protoporphyria.     

Surgical correction of pulmonic stenosis using transventricular pulmonic dilation valvuloplasty (Brock) in a dog

A two-month-old male long coated Chihuahua, which had symptoms including anorexia and decreased growth, was diagnosed with pulmonic valvular stenosis. Echocardiography showed the severe progression of pulmonary arterial velocity (6.29 m/s, Pressure gradient (PG); 158 mmHg). Transventricular pulmonic dilation valvuloplasty (Brock) was conducted, and stenosed valve was dilated by the oto forceps. Pleural effusion was observed postoperatively, however, it was diminished under the intensive care for five days after the surgery. The right ventricular systolic pressure was decreased to 60 mmHg in angiocardiography conducted two months postoperatively and the dog has kept a good general condition with recovered vigor and appetite.     

Dependence of self-tolerance on TRAF6-directed development of thymic stroma

The microenvironments of the thymus are generated by thymic epithelial cells (TECs) and are essential for inducing immune self-tolerance or developing T cells. However, the molecular mechanisms that underlie the differentiation of TECs and thymic compartmentalization are not fully understood. Here we show that deficiency in the tumor necrosis factor receptor-associated factor (TRAF) 6 results in disorganized distribution of medullary TECs (mTECs) and the absence of mature mTECs. Engraftment of thymic stroma of TRAF6(-/-) embryos into athymic nude mice induced autoimmunity. Thus, TRAF6 directs the development of thymic stroma and represents a critical point of regulation for self-tolerance and autoimmunity.