Species-dependent variations in erythrocyte membrane skeletal proteins

Two mammalian species (porcine and murine) have erythrocytes that are being widely used to study membrane protein synthesis and red cell aging. Erythrocytes of these species however, are significantly smaller than those of the human. Before results obtained from study of these red cells can be applied to human cells, the membrane skeleton of these species must be investigated to determine if the skeletal elements are equivalent. Both pig and mouse bands 4.1b were of lower molecular weight than human 4.1b, and the a/b ratio was lower. In each species, 4.1a and b were sequence-related phosphoproteins, and yielded substantially different one-dimensional peptide maps. Band 3 of pig and mouse erythrocytes had a higher molecular weight than human band 3 and also had differing one-dimensional peptide maps after limited proteolytic cleavage with three different enzymes. In each species, free band 3 and band 3 bound to the membrane skeleton had identical peptide maps. Other major membrane skeletal components (spectrin, actin, and bands 2.1 and 4.2) seem to be very similar in molecular weight in various species. These results demonstrate that the molecular weights and relative proportions of the membrane skeletal elements are species dependent.     

Identification of the molecular defect in the erythrocyte membrane skeleton of some kindreds with hereditary spherocytosis

We have localized the molecular alteration in the membrane skeleton of two of four kindreds with hereditary spherocytosis (HS) to an alteration in the spectrin-protein-4.1 interaction due to a defective spectrin molecule. The defective spectrin-protein-4.1 interaction in these kindreds (referred to as type I HS) leads to a weakened spectrin- protein-4.1-actin ternary complex, which in turn may lead to the friable membrane skeleton and suggested membrane instability related to this disorder. Type I HS spectrin binds approximately 63% as much protein-4.1 as normal spectrin (with equal affinity). This defect does not correlate with splenic function or erythrocyte age in the circulation. However, the approximately 37% reduction in binding of protein-4.1 to HS spectrin approaches the theoretical value of 50% expected in this autosomal dominant disorder. All other type I membrane skeletal interactions (spectrin-syndein, spectrin heterodimer- heterodimer, syndein-band-3) were found to be normal. It would appear therefore that the defective HS spectrin-protein-4.1 interaction in type I hereditary spherocytosis may be the primary molecular defect rather than a secondary phenomena.     

Type IIB Tampa: a variant of von Willebrand disease with chronic thrombocytopenia, circulating platelet aggregates, and spontaneous platelet aggregation

Type IIB von Willebrand disease is characterized by enhanced ristocetin- induced platelet aggregation and absence of large von Willebrand factor multimers from plasma. An alteration of the von Willebrand factor molecule resulting in increased reactivity with platelets appears to be the basis for these abnormalities. We have now identified a new variant of type IIB von Willebrand disease in a family in which the four affected members also have chronic thrombocytopenia, in vivo platelet aggregate formation, and spontaneous platelet aggregation in vitro. In spite of repeatedly prolonged bleeding times and persistent thrombocytopenia, their bleeding diathesis is only moderate.     

Artifacts seen during CT pelvimetry: implications for digital systems with scanning beams

During computed tomography (CT) pelvimetry of two pregnant women, bony abnormalities of the fetuses were noted on the scout images that were not confirmed at delivery. To explore the cause of these artifacts, specimen long bones were manipulated in various ways during CT scout imaging. Artifacts like those seen during in vivo imaging were found to be caused by motion of the object. The CT scout view is an example of an image produced by a digital system that uses a scanning beam. This type of digital system is being used for several types of body imaging including screening for scoliosis and chest radiography. Attention to motion artifacts should decrease diagnostic errors and aid further development of these systems.     

羽叶三七叶中甙类成分的研究

从羽叶三七叶中分离到十三种甙类成分,经FAB-MS,¹³CNMR谱,双照射¹HN-MR谱,¹H-¹H COSY谱及与标准品直接对照,证明十一种为已知化合物,分别为人参皂甙F₁(Ⅰ),F₂(Ⅱ),F₃(Ⅲ),R_g2(Ⅳ),R_a(Ⅴ),R_d(Ⅵ),R_b1(Ⅷ),R_b3(Ⅷ),24(S)-假人参甙F₁₁(Ⅸ),人参黄酮(Ⅹ)和珠子参甙F₁(Ⅺ);另外两种为新的达玛烷型皂甙,命名为羽叶三七甙F₁(Ⅻ)和F₂(ⅫⅠ),并确定其化学结构。同时修正珠子参甙F₃的结构。进一步阐明人参黄酮甙结构中的两个糖的连接方式。     

Splanchnic artery aneurysms and pseudoaneurysms: transcatheter embolization

Over the past 7 years, eight patients with splanchnic artery aneurysms and pseudoaneurysms were studied and treated. Transcatheter embolization resulted in occlusion of the lesions in all eight patients. Potentially risky and difficult surgery was avoided completely in four patients. Three patients had elective surgery at a later date when their condition was more stable. The remaining patient had definitive surgery after embolization. Transcatheter embolization should be the initial treatment of choice in splanchnic artery aneurysms and pseudoaneurysms.     

Bone marrow disorders: characterization with quantitative MR imaging

Thirty patients with various hematologic disorders and 15 healthy control subjects underwent quantitative magnetic resonance (MR) imaging of the lumbar spine with spin-echo techniques. Images of patients with infiltrative bone marrow disorders showed significantly more prolonged T1 times than those of control subjects (P less than .001). It was not possible to distinguish different diffuse infiltrative bone marrow disorders on the basis of T1 values. Aplastic anemia could be distinguished from normality because of significantly shortened T1 (P less than .001). A significant correlation was seen between T1 and bone marrow cellularity (r = .74, P less than .001). T2 was of no value in the characterization of bone marrow disorders. Quantitative MR imaging dose not improve the diagnostic potential of bone marrow imaging in the detection of diffuse marrow infiltrates.