急性早幼粒细胞白血病所致脑梗死1例报道并文献复习

目的 探讨急性早幼粒细胞白血病所致脑梗死患者的发病机制及临床特点。方法 对1例以急性早幼粒细胞白血病为病因的脑梗死患者的诊治过程进行报道,并结合相关文献分析急性早幼粒细胞白血病所致脑梗死的发病机制及临床特点。结果 脑梗死以急性早幼粒细胞白血病作为病因较少见,除了神经功能缺损的表现,还有凝血功能障碍的表现,发病机制包括肿瘤细胞淤积造成血管阻塞、促凝物质的表达增加、凝血级联反应的激活、血浆P-选择素的活性增强等。结论 应提高对以急性早幼粒细胞白血病为病因的脑梗死的认识,详细的病史和辅助检查有助于早期诊治,尤其是缺乏常见危险因素的年轻脑梗死患者,以免延误诊治。     

以脑梗死为首发表现的急性早幼粒细胞白血病一例报告

患者 ,男性 ,37岁。因急起左侧肢体乏力 ,言语不清 2ｄ就诊。在当地医院行头颅ＣＴ示“右基底节区及颞叶多发性脑梗死”。起病以来无头痛、恶心呕吐及意识改变。无发热、心悸、头昏、牙龈出血等症状。否认高血压、糖尿病、冠心病病史 ,无毒物及放射线接触史。入院查体 :血压 10 5 /70ｍｍＨｇ(14 0 /9 3ｋＰａ) ,全身无皮下出血点。右颌下扪及一黄豆大小淋巴结 ,肝脾肋下未及。专科检查 :意识清楚 ,言语含糊 ,左鼻唇沟变浅 ,口角右偏 ,伸舌左偏 ,颈软 ,左上肢肌力 2级 ,左下肢肌力 4级 ,左侧腱反射稍活跃 ,病理征阴性。入院后查 :ＷＢＣ 2 …     

中枢神经系统白血病患者脑脊液S-100b蛋白含量的测定

目的测定中枢神经系统白血病(CNSL)患者脑脊液(CSF)S-100b蛋白含量,并探讨其对CNSL患者脑损伤的评估价值.方法CNSL有神经系统损害症状患者(有症状组)14例,无神经系统损害症状患者(无症状组)22例和20例无神经系统疾病的外科腰麻患者(对照组),采用酶联免疫吸附试验双抗体夹心法检测CSFS-100b蛋白含量,同时进行CSF细胞学检查,并对36例CNSL患者CSF进行动态观察.结果有症状组CSFS-100b含量明显高于无症状组和对照组(均为P＜0.01),无症状组与对照组无显著性差异(P＞0.05);两组CNSL患者CSF小淋巴细胞比例均低于对照组(均为P＜001),有症状组与无症状组患者之间也有差异(P＜005);单核细胞比例有症状组患者最低,并与对照组有显著性差异(P＜0.05);动态检测发现,CSFS-100b蛋白含量随神经系统损害临床表现的加重或减轻而发生相应的变化.结论CNSL患者CSFS-100b蛋白含量与脑组织损伤的严重程度有关.     

老年精神分裂症脑脊液中tau蛋白与认知功能

目的：探讨脑脊液中ｔａｕ蛋白与认知功能缺损之间的关系。方法：精神分裂症组３７例,其他神经系统疾病组２６例,Ａｌｚｈｅｉｍｅｒ病组４５例,对精神分裂症患者评定阳性阴性症状量表,Ｆｕｌｄ物体记忆测验,言辞流畅测验,积木测验及数字广度测验。采用酶联免疫吸附分析检测３组患者中ｔａｕ蛋白浓度。     

精神分裂症认知功能损害特点及干预研究进展

本文阐述了精神分裂症患者的认知功能损害特点,从神经病理学和神经生化学等方面分析认知损害的可能机制,并对其干预方法作一概述.     

14-3-3蛋白对其他功能蛋白亚细胞定位的调控作用

14-3-3蛋白是所有真核细胞均表达的一组相对分子质量为(28～33)×103的酸性蛋白,该蛋白家族成员的氨基酸序列及功能等均具有高度保守性.已在哺乳动物的细胞中发现分别由不同基因编码的7种14-3-3蛋白亚型(β、γ、ε、σ、ξ、τ、η),它们主要存在于细胞质中,可自行组装成纯合型或杂合型二聚体.     

外周蛋白在中枢神经系统的生物学功能

<正>外周蛋白(peripherin)最初是在周围神经系统的感觉神经元内被发现,故命名为外周蛋白。后来,在中枢神经系统的某些感觉神经元和其他类别神经元,以及在PC12细胞内也发现存在有外周蛋白。中枢神经系统内外周蛋白的生物学功能还不清楚,可能与神经元对损伤的反应、神经再生和神经元变性等相关。一、外周蛋白的结构和特性中间纤维(intermediate filaments,IF)是一类直径8～11nm的中空管状纤维结构,它的直径介于微丝和微管之间。中间纤维蛋白分子由三部分组成:头部、尾部和中间的杆状区,中部杆     

信息与心理支持应用于急性白血病对其家属生理、心理及社会功能的影响

目的探究信息与心理支持应用于急性白血病患者对其家属生理、心理及社会功能的影响,并为这类患者最优化服务积累经验。方法选取我院血液科于2011年8月-2014年11月收治的86例急性白血病患者家属,利用随机数字表法进行分组,分别设为研究组和对照组,每组各43例。其中对照组开展常规护理,研究组则在对照组基础上实施信息与心理支持。记录两组患者家属分别于干预前1d及干预后第12w末接受世界卫生组织生活质量测定量表简表评估,并做好对比。结果两组患者家属在干预前1d的各维度评分差异无统计学意义(P>0.05)。两组生理功能、心理功能、社会功能、环境功能、生活质量总分、主观生活质量及主观健康状况评分均高于对照组,差异有统计学意义(P<0.05)。结论信息与心理支持应用于急性白血病家属,能有效提高其社会功能,改善其负性情绪,且能缓解其生理症状,取得较为可观的临床效果。     

β淀粉样蛋白对阿尔茨海默病转基因果蝇认知功能的影响

目的 探讨Aβ40、Aβ42、Aβ42Arc对阿尔茨海默病(AD)转基因果蝇认知功能的影响.方法 分别建立Aβ40、Aβ42、Aβ42Arc转基因AD果蝇模型.根据实验要求将果蝇分为4组,分别是野生果蝇对照组、Aβ40组、Aβ42组、Aβ42 Arc组.在整体动物水平上进行果蝇行为学研究,分别检测各组果蝇的嗅觉短期记忆能力、攀爬能力和平均寿命.结果 在巴甫洛夫嗅觉相关短期记忆测试、攀爬能力试验、平均寿命测试中,与对照组相比,3组转基因果蝇的嗅觉记忆能力、攀爬能力、平均寿命均明显降低.结论 Aβ的毒性作用会导致AD转基因果蝇认知功能下降.     

Wilson蛋白离子结合功能的研究

目的　研究Wilson蛋白 (ATP7B酶 )的离子结合功能 ,探讨锌剂治疗Wilson病的机制。方法　应用逆转录 聚合酶链反应 (RT PCR)扩增目的基因 ,GST基因融合载体表达并纯化融合蛋白 ,凝血酶酶切并收集目的蛋白 ,应用离子螯合层析和放射性锌印迹技术研究蛋白不同离子的结合能力。结果　ATP7B的离子结合能力为Cu(Ⅰ ) >Zn(Ⅱ ) >Ni(Ⅱ ) >Cu(Ⅱ ) ,并发现锌离子和铜离子对蛋白的结合具有竞争作用。结论　本实验证明了ATP7B以一价铜离子的形式行使其运铜功能 ,ATP7B不仅有结合铜离子的功能 ,而且有结合其他离子的功能 ;铜锌两种金属离子在蛋白结合位点的竞争作用可能是锌剂治疗WD的机制之一。     

Differential expression of alpha-synuclein isoforms in dementia with Lewy bodies

Dementia with Lewy bodies (DLB) is characterized by the widespread presence of Lewy bodies (LBs) in the brain. alpha-Synuclein, the main component of LBs, is expressed as two main isoforms (112 and 140), but little is known about their differential expression in the brain. We compared alpha-synuclein 112 and alpha-synuclein 140 expression levels in the prefrontal cortices of six DLB patients, eight Alzheimer disease (AD) patients, and six control subjects. Relative alpha-synuclein 112 and alpha-synuclein 140 expression levels were determined by real-time polymerase chain reaction with competimer technology using a LightCycler System. Whereas total alpha-synuclein levels were just marginally elevated in DLB in comparison with the other groups, alpha-synuclein 112 was seen to be markedly increased in DLB compared with AD cases and controls. In contrast, alpha-synuclein 140 levels were significantly diminished in both neurodegenerative disorders in comparison with controls. These results show differential overexpression of alpha-synuclein 112 in DLB, a finding that could be of importance in DLB pathogenesis.     

Actin sliding velocity on pure myosin isoforms from dystrophic mouse muscles

Duchenne muscular dystrophy (DMD) is a genetic disease characterized by skeletal muscle wasting and atrophy. Recent evidence suggests that the impaired skeletal muscle performance in DMD is not solely dependent on a loss of contractile muscle mass. In this study the myosin motor function of mdx and control (wildtype, WT) mice was compared using pure myosin isoforms in an “in vitro motility assay” (IVMA). Actin sliding velocity (Vf) on myosin 2B extracted from single muscle fibers of gastrocnemius muscles was significantly lower in mdx mice (3.48 ± 0.13 μm/s, n = 18) than in WT mice (4.02 ± 0.19 μm/s, n = 10). No difference in Vf was found between myosin 1 extracted from soleus muscles of mdx (0.84 ± 0.04 μm/s, n = 13) and of WT (0.89 ± 0.04 μm/s, n = 10). The results suggest that the dystrophic process alters myosin molecular function, and this contributes to the functional impairment in dystrophic muscles. Muscle Nerve 40: 249–256, 2009     

Three isoforms of human myelin basic protein: Purification and structure

Myelin basic protein (MBP) occurs in multiple forms. Three of these isoforms from human MBP (HMBP) have been highly purified. HMBP, component 1 (18.5 kDa HMNP-1), was purified by ion-exchange chromatography at pH 10.6 in 2 M urea. During this ion-exchange chromatography, a fraction (Fraction 3), which contained HMBP component 3 (monophosphorylated or deamidated 18.5 kDa) and 17.2 kDa HMBP, was collected and further purified by fast protein liquid chromatography, which separated 17.2 kDa HMBP and HMDP component 3. When the latter was subjected to limited thrombic digestion, all of HMBP component 3 not phosphorylated at theonine 98 was cleaved. This digestion mixture was separated on Sephadex, and yielded pure component 3, monophosphorylated at theonine 98 (HMBP 3pT98), for which phosphate analysis yielded approximately 1 mole P/mole protein, and NMR showed only one phosphorylation site present. Circular dichroism (CD) studies were carried out on dilute solutions of HMBP-1 (18.5 kDa), 17.2 kDa HMBP, and HMBP3pT98 (phosphorylated 18.5 kDa). The CD spectrum of HMBP-1 was similar to that reported for rabbit MBP-1 and bovine MBP-1, but the spectra of 17.2 kDa HMBP and HMBP 3pT98 were distinctly different from HMBP-1. When analyzed by best-fit computations, 17.2 kDa HMBP showed about a 9% increase of ordered structure, and a greater increase, about 12%, was estimated for HMBP3pT98, attributable to β-structure and β turn. © 1995 Wiley-Liss, Inc.     

Channel activity of deamidated isoforms of prion protein fragment 106-126 in planar lipid bilayers

Using the lipid bilayer technique, we have found that age-related derivatives, PrP[106-126] (L-Asp108) and PrP[106-126] (L-iso-Asp108), of the prion protein fragment 106-126 (PrP[106-126] (Asn108)) form heterogeneous ion channels. The deamidated isoforms, PrP[106-126] (L-Asp108) and PrP[106-126] (L-iso-Asp108), showed no enhanced propensity to form heterogeneous channels compared with PrP[106-126] (Asn108). One of the PrP[106-126] (L-Asp108)- and PrP[106-126] (L-iso-Asp108)-formed channels had three kinetic modes. The current-voltage (I-V) relationship of this channel, which had a reversal potential, E(rev), between -40 and -10 mV close to the equilibrium potential for K+ (E(K)-35 mV), exhibited a sigmoidal shape. The value of the maximal slope conductance (g(max)) was 62.5 pS at positive potentials between 0 and 140 mV. The probability (P(o)) and the frequency (F(o)) of the channel being open had inverted and bell-shaped curves, respectively, with a peak at membrane potential (V(m)) between -80 and +80 mV. The mean open and closed times (T(o) and T(c)) had inverted bell-shaped curves. The biophysical properties of PrP[106-126] (L-Asp108)- and PrP[106-126] (L-iso-Asp108)-formed channels and their response to Cu(2+) were similar to those of channels formed with PrP[106-126] (Asn108). Cu(2+) shifted the kinetics of the channel from being in the open state to a "burst state" in which rapid channel activities were separated by long durations of inactivity. The action of Cu(2+) on the open channel activity was both time-dependent and voltage-dependent. The fact that Cu(2+) induced changes in the kinetics of this channel with no changes in the conductance of the channel indicated that Cu(2+) binds at the mouth of the channel. Consistently with the hydrophilic and structural properties of PrP[106-126], the Cu(2+)-induced changes in the kinetic parameters of this channel suggest that the Cu(2+) binding site could be located at M(109) and H(111) of this prion fragment.     

Coexpression of myosin isoforms in muscle of patients with neurogenic disease

Three well-characterized antimyosin heavy chain monoclonal antibodies (McAbs) were used as immunocytochemical reagents to study myosin isoform expression in relationship to adenosine triphosphatase (ATPase) defined fiber types in human muscle. The biopsy specimens were from patients with neurogenic muscle disease whose muscle exhibited fiber type grouping and group atrophy. The use of McAbs revealed heretofore unrecognized coexpression of multiple myosin isoforms in selected fibers in the pathologic samples which was not apparent with ATPase reactions and not present in normal muscle. The fibers containing multiple myosin isoforms were probably undergoing neurally directed fiber type transformation. Furthermore, a small population of fibers in neurogenic specimens expressed a "prenatal" myosin signifying the presence of regenerating fibers. We also demonstrated immunocytochemical evidence of the persistence of adult slow myosin in denervated mature human skeletal muscle despite the reputed necessity of innervation for maintenance of expression of this myosin isoform proffered by others.     

Protein kinase Calpha and beta1 isoforms are regulators of alpha-secretory proteolytic processing of amyloid precursor protein in vivo

We have recently shown that in utero treatment of guinea pigs with the DNA methylating substance methylazoxymethanol acetate (MAM) results in neocortical microencephalopathy, increased protein kinase C (PKC) activity and altered processing of the amyloid precursor protein (APP) in neocortex of offspring. Here we show that PKCα and PKCβ1 are the key regulators of α‐secretory APP processing in guinea pig neocortex under these experimental conditions in vivo. This conclusion is based on the selective translocation of PKCα and PKCβ1 isoforms to the cell membrane in MAM‐treated guinea pigs, as revealed by Western blot analysis and by immunocytochemistry. Additionally, we observed that [³H]phorbol ester binding to protein kinase C increased by 38% and enhanced basal PKC activity by 58% in the neocortex of microencephalic guinea pigs. Inhibition of PKCα/PKCβ1 by Gö6976 abolished this difference, suggesting that constitutive overactivation of these PKC isoforms accounts for the increase in total PKC activity. We also observed a strong positive correlation between levels of α‐secretase‐processed APP and PKC activity in the neocortex of individual animals, providing further evidence for a significant role of classical PKC isoforms in nonamyloidogenic APP processing.     

Infantile cortical measles inclusion body encephalitis during combined treatment of acute lymphoblastic leukemia

Summary A case of cortical measles inclusion body encephalitis occuring in a boy aged 6 years 7 months, 4 months after uncomplicated measles is reported. The child was undergoing combined treatment for acute lymphoblastic leukemia. He was in primary remission for 2 years. The neuropathological findings are characterized by necrosis, eosinophilic nuclear and cytoplasmic inclusion bodies in the neuronal and glial cells within the cerebral cortex and basal ganglia. Nucleocapsides of paramyxoviruses were detected in the nuclear inclusion bodies of both cell types.

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Zusammenfassung Es wird über eine akute corticale Masern-Einschlußkörper-Encephalitis berichtet, die 4 Monate nach Erkrankung an Masern bei einem 6 7/12 jährigen Jungen mit kombiniert therapierter saure-Phosphatasepositiver Lymphoblasten-Leukose während zweijähriger Primärremission auftrat. Die neuropathologischen Befunde sind durch Nervenzelluntergang, eosinophile Kern- und Cytoplasmaeinschlußkörper in Nerven- und Gliazellen im Bereich der Hirnrinde und Stammganglien charakterisiert. In den Kerneinschlußkörpern wurden Nukleokapside von Paramyxoviren nachgewiesen.