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排序方式: 共有491条查询结果,搜索用时 15 毫秒
1.
Cardiac calsequestrin (CASQ2) contributes to intracellular Ca2+ homeostasis by virtue of its low-affinity/high-capacity Ca2+ binding properties, maintains sarcoplasmic reticulum (SR) architecture and regulates excitation–contraction coupling, especially or exclusively upon β-adrenergic stimulation. Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease associated with cardiac arrest in children or young adults. Recessive CPVT variants are due to mutations in the CASQ2 gene. Molecular and ultra-structural properties were studied in hearts of CASQ2R33Q/R33Q and of CASQ2−/− mice from post-natal day 2 to week 8. The drastic reduction of CASQ2-R33Q is an early developmental event and is accompanied by down-regulation of triadin and junctin, and morphological changes of jSR and of SR-transverse-tubule junctions. Although endoplasmic reticulum stress is activated, no signs of either apoptosis or autophagy are detected. The other model of recessive CPVT, the CASQ2−/− mouse, does not display the same adaptive pattern. Expression of CASQ2-R33Q influences molecular and ultra-structural heart development; post-natal, adaptive changes appear capable of ensuring until adulthood a new pathophysiological equilibrium.  相似文献   
2.
Classical homocystinuria is the most commonly inherited disorder of sulfur metabolism, caused by the genetic alterations in human cystathionine beta-synthase (CBS) gene. In this study, we present comprehensive clinical findings and the genetic basis of homocystinuria in a cohort of Turkish patients. Excluding some CBS mutations, detailed genotype–phenotype correlation for different CBS mutations has not been established in literature. We aimed to make clinical subgroups according to main clinical symptoms and discussed these data together with mutational analysis results from our patients. Totally, 16 different mutations were identified; twelve of which had already been reported, and four are novel (p.N93Y, p.L251P, p.D281V and c.829−2A>T). The probands were classified into three major groups according to the clinical symptoms caused by these mutations. A psychomotor delay was the most common diagnostic symptom (n = 12, 46.2% neurological presentation), followed by thromboembolic events (n = 6, 23.1% vascular presentation) and lens ectopia, myopia or marfanoid features (n = 5, 19.2% connective tissue presentation). Pyridoxine responsiveness was 7.7%; however, with partial responsive probands, the ratio was 53.9%.  相似文献   
3.
廖永伯  张琪  丁金凤 《生理学报》1991,43(4):368-375
培养的卒中型自发性高血压大鼠(SHR_(sp))及其对照 WKY 大鼠主动脉平滑肌细胞(VSMC)上存在心房钠尿肽(ANP)的特异性受体,它们与~(125)I-ANP 的最大结合量(B_(max))是:SHR_(sp)3.65±0.13和 WKY 1.89±0.09 pmol/mg pr(P<0.01);解离平衡常数(Kd)值分别是72.6±10.2和42.0±4.8×10~(-12)mol/L(P<0.01)。 两种细胞内介导舒血管作用的第二信使、环磷酸乌苷(cGMP)的基础浓度无显著差异,对相同剂量 ANP 刺激引起 cGMP 分别增加139(SHRsp)和271(WKY)倍。可见 SHRsp 的 VSMC ANP 受体数量虽比 WKY大鼠增多,但对相同剂量 ANP 引起的 cGMP 增加反应及 ANP 受体的亲和力均显著降低。高盐培养液孵育24h 后,细胞表面 ANP 受体的亲和力改变不明显,但受体数量下调,SHRsp 和 WKY 大鼠分别降至对照的34.8±8.2%和38.6±9.4%,细胞对 ANP 引起的 cGMP增加反应明显降低,且均以 SHR_(sp)较显著。提示后两种变化可能在高盐促进血压升高的机制中起作用。  相似文献   
4.
用基因定点诱变技术,删除了pO_1α ANF表达质粒中的33对碱基,使人α型心钠素结构基因直接与大肠杆菌分泌型表达质粒pIN-Ⅲ-OmPA中的信号肽酶切位点编码区相连,构成天然人α型心钠素的表达质粒pANF,在IPTG诱导下表达28肽的天然人α型心钠素。纯化后的表达产物具有天然心钠素的放免活性和很强的舒张血管的生物活性。  相似文献   
5.
Miller LJ 《Zoo biology》2012,31(2):242-248
Many publications within the field of zoo animal welfare have stated the importance of decreasing stereotypic behavior (e.g., pacing) to help ensure a positive visitor experience. The idea behind these statements is that visitors want to see animals engaged in natural behavior. Additionally, it is thought that watching an animal exhibit species-appropriate behavior could help increase a visitor's connection to wildlife and ultimately their interest in conservation. However, until recently, no information was available to validate such statements. The purpose of this research was to examine people's reaction to viewing an animal engaged in pacing behavior. Participants were randomly selected to fill out a survey after watching a short video of either a tiger pacing or resting (control). Results indicate that having viewed a tiger pacing significantly decreases people's perception of the level of care animals receive at that facility. In addition, people's interest in supporting zoos decreased as a result of viewing this behavior. Results are discussed from an animal welfare, business, and conservation perspective.  相似文献   
6.
Cardiac resynchronization therapy device (CRT-P and CRT-D) implantation has increased tremendously with increasing operator experience, eligible patients and expansion of indications. Refinements in devices and algorithms now aid physicians to improve biventricular pacing and optimize CRT. We report a case in which an interesting device program was used to achieve biventricular pacing after repeated dislodgement of the atrial lead in a patient implanted with CRT-D.  相似文献   
7.
Lipin functions in mammalian phospholipid biosynthesis through its phosphatidate phosphohydrolase 1 (PAP1) activity. Here, we studied cardiac PAP1 activity and lipin expression ex vivo in 8-month-old Zucker diabetic fatty (ZDF) rats and humans with type 2 diabetes mellitus undergoing open heart surgery for coronary bypass grafting. Compared to non-diabetic littermates (ZDF-fa/+), left ventricular PAP1 activity was 29% lower in diabetic ZDF-fa/fa rats. Left ventricular PAP1 activities were 2.1-fold (ZDF-fa/fa) and 3.6-fold (ZDF-fa/+) higher than the respective atrial activities, indicating marked differences in cardiac distribution of PAP1. PAP1 activity was highly related with cardiac lipin-1 and lipin-3 mRNA expression in ZDF rats (= 0.99 and 0.96). Consistent with the findings in experimental animals, human atrial tissue displayed PAP1 activity that was 33% lower in those having diabetes than in non-diabetic controls. Accordingly, atrial lipin-1 and lipin-3 mRNA expression in diabetic patients was 50% and 59% lower as in non-diabetic patients, respectively. Insulin therapy increased both PAP1 activity and lipin mRNA expression in diabetic patients. We conclude that suppression of cardiac PAP1 activity/lipin expression may contribute to metabolic dysfunction of the diabetic heart.  相似文献   
8.
9.
The stereotyped pacing shown by the two Amur tigers in the Zurich Zoo was hypothesized as being caused by permanently frustrated appetitive foraging behavior. Several electrically controlled feeding boxes were installed and access to each box was possible only twice a day for 15 min at semi‐random times. The boxes had to be opened actively by the tigers. Two trials were carried out: one with solitary confinement, and one with paired confinement. During box feeding, the female's stereotyped pacing was significantly reduced from 16% (solitary confinement, conventional feeding) and 7% (paired confinement, conventional feeding) to 1% (solitary confinement) and less than 0.01% (paired confinement) of the daily observed time. The female's sleeping increased significantly in both solitary and paired confinement. The male only showed a significant reduction in stereotyped pacing behavior when kept with the female (conventional feeding: 10%; box feeding: <0.01% of the daily observed time). On days with a box‐feeding regime in paired confinement, the male spent 25% (83 min) of the observed time with active behavior at the feeding boxes. The results support the hypothesis that permanently frustrated appetitive foraging behavior causes stereotyped pacing in adult tigers. Zoo Biol 21:573–584, 2002. © 2002 Wiley‐Liss, Inc.  相似文献   
10.
Transvenous endocardial pacing through classical implantation of a pace/sensing lead in the right ventricle is strictly contraindicated in patients with a mechanical tricuspid valve. Usually permanent pacing is achieved by an epimyocardial surgical approach. We hereby describe the implantation of a single site left ventricle pacing lead in the anterior interventricular vein in a 60 year-old woman with symptomatic bradycardia, permanent atrial fibrillation, and mechanical tricuspid valve. The described use of left ventricle pacing through a coronary vein lead, in a patient with favorable venous anatomy, provided (through a minimal invasive approach) effective with a low and stable threshold.  相似文献   
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