检索结果-内蒙古大学图书馆

Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

引用

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 1999年第13期96卷 7388-7393页

作者： Karpuj, MV Garren, H Slunt, H Price, DL Gusella, J Becher, MW Steinman, L Stanford Univ Dept Neurol Sci Stanford CA 94305 USA Weizmann Inst Sci IL-76100 Rehovot Israel Harvard Univ Sch Med Neurogenet Lab Charlestown MA 02129 USA Johns Hopkins Univ Sch Med Dept Pathol Div Neuropathol Baltimore MD 21205 USA Univ New Mexico Hlth Sci Ctr Dept Pathol Albuquerque NM 87131 USA

The protein huntingtin (htt), aggregated in neuronal nuclear inclusions, is pathognomonic of Huntington's disease (HD), Constructs, translated in I vitro from the N terminus of htt, containing either polyQ23 from a normal individual, or polyQ41 or polyQ67 from an HD patient, were all soluble. Transglutaminase (TGase) crosslinked these proteins, and the aggregations did not have the staining properties of amyloid, More TGase-catalyzed aggregates formed when the polyglutamine domain of htt exceeded the pathologic threshold of polyQ36. Furthermore, shorter htt constructs, containing 135 aa or fewer, formed more aggregates than did larger htt constructs. TGase activity in the HD brain was increased compared with the control, with notable increases in cell nuclei, The increased TGase activity was brain specific. In lymphoblastoid cells from HD patients, TGase activity Has decreased. TGase-mediated crosslinking of htt may be involved in the formation of the nonamyloidogenic nuclear inclusions found in the HD brain, The staining properties of nuclear inclusions in the HD brain revealed that they were not amyloid.

关键词：脑/代谢脑/超微结构细胞核/代谢交联试剂二聚作用酶激活杭廷顿病/代谢神经组织蛋白质类/代谢核蛋白质类/代谢肽碎片/代谢转谷酰胺酶/代谢成年人老年人老年人 80以上人类男(雄)性中年人

来源：评论

学校读者我要写书评

暂无评论

Protein binding of a DRPLA family through arginine-glutamic acid dipeptide repeats is enhanced by extended polyglutamine

引用

HUMAN MOLECULAR GENETICS 2000年第9期9卷 1433-1442页

作者： Yanagisawa, H Bundo, M Miyashita, T Okamura-Oho, Y Tadokoro, K Tokunaga, K Yamada, M Natl Childrens Med Res Ctr Setagaya Ku Tokyo 1548509 Japan Univ Tokyo Grad Sch Med Dept Human Genet Tokyo 1138654 Japan

Dentatorubral-pallidoluysian atrophy (DRPLA) is one of the hereditary neurodegenerative disorders caused by expansion of CAG/glutamine repeats. To investigate the normal function of the DRPLA gene and the pathogenic mechanism of neuron death in specific areas of the brain, we isolated and analyzed a gene that shares a notable motif with DRPLA, arginine-glutamic acid (RE) dipeptide repeats. The gene isolated, designated RERE, has an open reading frame of 1566 amino acids, of which the C-terminal portion has 67% homology to DRPLA, whereas the N-terminal portion is distinctive. RERE also contains arginine-aspartic acid (RD) dipeptide repeats and putative nuclear localization signal sequences, but no polyglutamine tracts. RERE is expressed at a low level in most tissues examined. Immunoprecipitation and in vitro binding assays demonstrate that the DRPLA and RERE proteins bind each other, for which one of the RE repeats has a primary role, and extended polyglutamine enhances the binding. With engineered constructs fused with a tag, the RERE protein localized predominantly in the nucleus. Moreover, when RERE is overexpressed, the distribution of endogenous DRPLA protein alters from the diffused to the speckled pattern in the nucleus so as to co-localize with RERE. More RERE protein is recruited into nuclear aggregates of the DRPLA protein with extended polyglutamine than into those of pure polyglutamine. These results reveal a function for the DRPLA protein in the nucleus and the RE repeat in the protein-protein interaction.

关键词：氨基酸序列精氨酸/代谢印迹法蛋白质脑/代谢载体蛋白质类/化学载体蛋白质类/遗传学细胞核/代谢交联试剂/代谢细胞质/代谢 DNA 互补/代谢谷氨酸/代谢绿色荧光蛋白质类 HeLa细胞发光蛋白质类/代谢分子序列数据神经组织蛋白质类/遗传学神经组织蛋白质类/代谢开放读码框架肽类/代谢质粒沉淀素试验蛋白质结合重组融合蛋白质类/代谢序列同源性氨基酸信号传导组织分布转染人类

来源：评论

学校读者我要写书评

暂无评论

Development of neuron-neuron synapses

引用

CURRENT OPINION IN NEUROBIOLOGY 2000年第1期10卷 125-131页

作者： Lee, SH Sheng, M Massachusetts Gen Hosp Dept Neurobiol Boston MA 02114 USA Massachusetts Gen Hosp Howard Hughes Med Inst Boston MA 02114 USA Harvard Univ Sch Med Boston MA 02114 USA

Our understanding of neuronal synapse development has advanced in recent years. The development of glycinergic synapses appears to depend on gephyrin and glycine receptor activity. Molecular characterization of the structure and development of glutamatergic synapses is in progress, but the underlying mechanisms remain unclear. Activity-dependent mechanisms and specific molecules that regulate the morphological development of dendritic spines have recently been identified.

关键词：树突/代谢运动神经肌肉终板/化学运动神经肌肉终板/代谢神经生长因子类/生理学神经生长因子类/分泌神经组织蛋白质类/代谢神经系统/细胞学神经系统/生长和发育神经系统/代谢神经元/细胞学神经元/代谢受体谷氨酸/代谢突触/化学突触/代谢突触/生理学动物人类

来源：评论

学校读者我要写书评

暂无评论

Neuronal control of locomotion in C-elegans is modified by a dominant mutation in the GLR-1 ionotropic glutamate receptor

引用

NEURON 1999年第2期24卷 347-361页

作者： Zheng, Y Brockie, PJ Mellem, JE Madsen, DM Maricq, AV Univ Utah Dept Biol Salt Lake City UT 84112 USA

How simple neuronal circuits control behavior is not well understood at the molecular or genetic level. In Caenorhabditis elegans, foraging behavior consists of long, forward movements interrupted by brief reversals. To determine how this pattern is generated and regulated, we have developed novel perturbation techniques that allow us to depolarize selected neurons in vivo using the dominant glutamate receptor mutation identified in the Lurcher mouse. Transgenic worms that expressed a mutated C. elegans glutamate receptor in interneurons that control locomotion displayed a remarkable and unexpected change in their behavior-they rapidly alternated between forward and backward coordinated movement. Our findings suggest that the gating of movement reversals is controlled in a partially distributed fashion by a small subset of interneurons and that this gating is modified by sensory input.

关键词：氨基酸序列/遗传学动物基因修饰/遗传学动物基因修饰/生理学细胞凋亡/生理学秀丽新小杆线虫/遗传学秀丽新小杆线虫/生理学漂亮新小杆线虫蛋白质类半胱氨酸天冬氨酸蛋白酶1/遗传学基因表达/生理学基因显性/遗传学中间神经元/生理学离子通道/代谢机械感受器/生理学膜蛋白质类/遗传学膜蛋白质类/代谢分子序列数据运动活动/生理学突变/生理学神经组织蛋白质类/遗传学神经组织蛋白质类/代谢神经通路/生理学神经元/生理学表型启动区遗传/生理学受体 AMPA 受体谷氨酸感觉/生理学动物

来源：评论

学校读者我要写书评

暂无评论

Mouse VAP33 is associated with the endoplasmic reticulum and microtubules

引用

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 2000年第3期97卷 1101-1106页

作者： Skehel, PA Fabian-Fine, R Kandel, ER Natl Inst Med Res Div Neurophysiol London NW7 1AA England Open Univ Dept Biol Sci Milton Keynes MK7 6AA Bucks England Columbia Univ Howard Hughes Med Inst Ctr Neurobiol & Behav New York NY 10032 USA

VAMP/synaptobrevin is a synaptic vesicle protein that is essential for neurotransmitter release. Intracellular injection of antisera against the Aplysia californica VAMP/synaptobrevin-binding protein ApVAP33 inhibited evoked excitatory postsynaptic potentials (EPSPs) in cultured cells, suggesting that this association may regulate the function of VAMP/synaptobrevin. We have identified and characterized a mouse homologue of ApVAP33, mVAP33. The overall domain structure of the proteins is conserved, and they have similar biochemical properties. mVAP33 mRNA is detectable in all mouse tissues examined, in contrast to the more restricted expression seen in A. californica, We analyzed the cellular distribution of mVAP33 protein in brain slices and cultured cortical cells by light and electron microscopy, Although present at higher levels in neurons, immunoreactivity was detected throughout both neurons and glia in a reticular pattern similar to that of endoplasmic reticulum-resident proteins. mVAP33 does not colocalize with VAMP/synaptobrevin at synaptic structures, but expression overlaps with lower levels of VAMP/synaptobrevin in the soma, Ultrastructural analysis revealed mVAP33 associated with microtubules and intracellular vesicles of heterogeneous size. In primary neuronal cultures, large aggregates of mVAP33 are also detected in short filamentous structures, which are occasionally associated with intracellular membranes. There is no evidence for accumulation of mVAP33 on synaptic vesicles or at the plasma membrane. These data suggest that mVAP33 is an endoplasmic-reticulum-resident protein that associates with components of the cytoskeleton. Any functional interaction between mVAP33 and VAMP/synaptobrevin, therefore, most likely involves the delivery of components to synaptic terminals rather than a direct participation in synaptic vesicle exocytosis.

关键词：氨基酸序列海螺/免疫学载体蛋白质类/免疫学载体蛋白质类/代谢细胞培养的内质网/代谢兴奋性突触后电位荧光抗体技术间接海马/代谢海马/超微结构免疫组织化学大分子物质膜蛋白质类/分析膜蛋白质类/免疫学膜蛋白质类/代谢微管/代谢分子序列数据神经组织蛋白质类/免疫学神经组织蛋白质类/代谢神经胶质/代谢神经元/代谢器官特异性 R-SNARE蛋白质类 RNA 信使/分析重组融合蛋白质类/免疫学重组融合蛋白质类/代谢序列比对序列同源性氨基酸种特异性突触小泡/代谢突触小泡/超微结构膜泡运输蛋白质类动物人类小鼠大鼠

来源：评论

学校读者我要写书评

暂无评论

DIRECTIONALITY AND RATE OF ASSEMBLY OF CHICK BRAIN TUBULIN ONTO PIECES OF NEUROTUBULES, FLAGELLAR AXONEMES, AND BASAL BODIES

引用

ANNALS OF THE NEW YORK ACADEMY OF SCIENCES 1975年第JUN30期253卷 147-177页

作者： ROSENBAUM, JL BINDER, LI GRANETT, S DENTLER, WL SNELL, W SLOBODA, R HAIMO, L YALE UNIV DEPT BIOL NEW HAVEN CT 06520 USA

来源：评论

学校读者我要写书评

暂无评论

Mutagenesis reveals a role for ABP/GRIP binding to GluR2 in synaptic surface accumulation of the AMPA receptor

引用

NEURON 2000年第2期27卷 313-325页

作者： Osten, P Khatri, L Perez, JL Köhr, G Giese, G Daly, C Schulz, TW Wensky, A Lee, LM Ziff, EB Max Planck Inst Med Res Dept Mol Neurobiol D-69120 Heidelberg Germany NYU Sch Med Dept Biochem Howard Hughes Med Inst New York NY 10016 USA Max Planck Inst Med Res Dept Biomed Opt D-69120 Heidelberg Germany

We studied the role of PDZ proteins GRIP, ABP, and PICK1 in GluR2 AMPA receptor trafficking. An epitope-tagged MycGluR2 subunit, when expressed in hippocampal cultured neurons, was specifically targeted to the synaptic surface. With the mutant MycGluR2 Delta 1-10, which lacks the PDZ binding site, the overall dendritic intracellular transport and the synaptic surface targeting were not affected. However, over time, MycGluR2 Delta 1-10 accumulated at synapses significantly less than MycGluR2. Notably, a single residue substitution, S880A, which blocks binding to ABP/GRIP but not to PICK1, reduced synaptic accumulation to the same extent as the PDZ site truncation. We conclude that the association of GluR2 with ABP and/or GRIP but not PICK1 is essential for maintaining the synaptic surface accumulation of the receptor, possibly by limiting its endocytotic rate.

关键词：衔接蛋白质类信号转导结合部位/遗传学载体蛋白质类/代谢细胞培养的海马/细胞学海马/代谢诱变定点神经组织蛋白质类/代谢神经元/细胞学神经元/代谢核蛋白质类/代谢蛋白质结构三级/遗传学原癌基因蛋白质c-myc/遗传学大鼠 Sprague-Dawley 受体 AMPA/遗传学受体 AMPA/代谢重组融合蛋白质类/遗传学重组融合蛋白质类/代谢 Sindbis病毒/遗传学突触膜/代谢动物大鼠

来源：评论

学校读者我要写书评

暂无评论

Adipocyte expression of a neuronal RNA-binding protein

引用

ADVANCES IN ENZYME REGULATION, VOL 41 2001年第1期41卷 209-220页

作者： Qi, C Karkut, C Lombardi, N Pruett, A Tenney, R Wheeler, J Pekala, PH E Carolina Univ Brody Sch Med Dept Biochem Greenville NC 27858 USA

Mel-N1 (murine embryonic lethal abnormal vision [ELAV]-1), a mammalian homolog of Drosophila ELAV, is an mRNA binding protein of the RNA Recognition Motif family. Studies have supported a role for both Mel-N1, and its splice variant, Mel-N2, in mRNA metabolism. Mel-N1 has been characterized exclusively as a neuronal protein, while its human homolog, Hel-N1, is predominantly expressed in neuronal tissue but exhibits minor levels of expression in both ovaries and testies. Our current work expands the potential function of these proteins through demonstration of their expression in adipose tissue and the 3T3-L1 cells. Cloning and sequencing studies have demonstrated the expression of Mel-N1 and two splice variants Mel-N2a and -N2b. N2a and N2b differ from N1 by the deletion of 45 and 48 bases, respectively. N2a and N2b differ from each other by three bases and the absence of a single serine at the protein level. While all three forms are expressed in both the 3T3-L1 preadipocytes and adipocytes there is a marked down regulation of N1 expression as the cells differentiate. Mel-N2b, however, is the predominant form expressed in both preadipocytes and adipocytes. Expression of both Mel-N1 and Mel-N2 was observed in murine white adipose tissue, supporting the premise that expression of these proteins in the 3T3-L1 cells is not an artifact of cell culture. Based on these data as well as our previous studies, we suggest that in adipocytes Mel-N1 and N2 may control gene expression through alteration of mRNA stability and rates of translation initiation through the specific binding to target mRNAs.

关键词：脂细胞/代谢选择性剪接氨基酸序列碱基序列细胞系 DNA 互补/代谢葡萄糖转运体1型 Hu副肿瘤性脑脊髓炎抗原配体分子序列数据单糖转运蛋白质类/代谢神经组织蛋白质类/化学神经组织蛋白质类/遗传学神经组织蛋白质类/代谢神经元/代谢蛋白质结合蛋白质生物合成 RNA结合蛋白质类/化学 RNA结合蛋白质类/代谢序列同源性氨基酸序列同源性核酸动物人类小鼠

来源：评论

学校读者我要写书评

暂无评论

Protein 7B2 is essential for the targeting and activation of PC2 into the regulated secretory pathway of rMTC 6-23 cells

引用

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 1999年第2期257卷 473-479页

作者： Barbero, P Kitabgi, P Univ Nice Sophia Antipolis CNRS Inst Pharmacol Mol & Cellulaire F-06560 Valbonne France

Among the prohormone convertases, PC2 is unique in that it specifically binds to the neuroendocrine-specific protein 7B2 in the endoplasmic reticulum (ER) and is activated late in the regulated secretory pathway of neuroendocrine cells. Several roles, sometimes contradictory, have been suggested for 7B2 with regard to PC2 cellular fate. Thus, 7B2 was proposed to act as a PC2 chaperone in the ER, or to facilitate 7B2 transport from the ER to the trans-Golgi network and to be necessary for proPC2 activation, or to inhibit PC2 enzymatic activity until the latter reaches the secretory granules. To gain insight into the function of 7B2, we sought to block its expression in PC2-expressing endocrine cells using antisense strategies. We have previously shown that the endocrine rMTC 6-23 cell line expresses PC2 and that the enzyme is responsible for the processing of pro-neurotensin/neuromedin N (proNT/NN). Here, we show that rMTC 6-23 cells express 7B2 and that the protein was coordinately induced with PC2 and proNT/NN by dexamethasone. Stable transfection of rMTC 6-23 cells with 7B2 antisense cDNA led to a marked reduction (>90%) in 7B2 levels. ProPC2 was expressed to normal levels and cleaved to yield a PC2 form that was constitutively released, was not stored within secretory granules and was unable to process proNT/NN. We conclude that 7B2 is essential for the sorting and activation of PC2 into the regulated secretory pathway of endocrine cells. (C) 1999 Academic Press.

关键词：印迹法蛋白质细胞系细胞质颗粒/代谢细胞质颗粒/分泌 DNA 反义/遗传学地塞米松/药理学减量调节内分泌腺/细胞学内分泌腺/分泌内质网/酶学内质网/代谢内质网/分泌酶激活酶诱导/药物作用免疫组织化学分子量神经组织蛋白质类/遗传学神经组织蛋白质类/代谢神经内分泌蛋白质7B2 神经降压肽/代谢垂体激素类/遗传学垂体激素类/代谢前蛋白转化酶2 蛋白质前体/代谢枯草杆菌蛋白酶类/代谢枯草杆菌蛋白酶类/分泌转染动物大鼠

来源：评论

学校读者我要写书评

暂无评论

CNS myelin and Sertoli cell tight junction strands are absent in Osp/Claudin-11 null mice

引用

CELL 1999年第6期99卷 649-659页

作者： Gow, A Southwood, CM Li, JS Pariali, M Riordan, GP Brodie, SE Danias, J Bronstein, JM Kachar, B Lazzarini, RA CUNY Mt Sinai Med Ctr Brookdale Ctr Dev & Mol Biol New York NY 10029 USA CUNY Mt Sinai Med Ctr Dept Ophthalmol New York NY 10029 USA NICHHD Sect Struct Cell Biol NIH Bethesda MD 20892 USA Univ Calif Los Angeles Sch Med Dept Neurol Los Angeles CA 90095 USA

Oligodendrocyte-specific protein (OSP)/claudin-11 is a recently identified transmembrane protein found in CNS myelin and testis with unknown function. Herein we demonstrate that Osp null mice exhibit both neurological and reproductive deficits: CNS nerve conduction is slowed, hindlimb weakness is conspicuous, and males are sterile. Freeze fracture reveals that tight junction intramembranous strands are absent in CNS myelin and between Sertoli cells of mutant mice. Our results demonstrate that OSP is the mediator of parallel-array tight junction strands and distinguishes this protein from other intrinsic membrane proteins in tight junctions. These novel results provide direct evidence of the pivotal role of the claudin family in generating the paracellular physical barrier of tight junctions necessary for spermatogenesis and normal CNS function.

关键词：脑/细胞学脑/代谢冷冻断裂基因表达调控发育期类人猿亚目后肢/生长和发育不育男(雄)性/代谢不育男(雄)性/病理学膜蛋白质类/遗传学膜蛋白质类/代谢小鼠基因敲除显微镜检查共焦显微镜检查电子髓鞘/代谢神经组织蛋白质类/遗传学神经组织蛋白质类/代谢少突神经胶质/细胞学少突神经胶质/代谢塞尔托利细胞/代谢塞尔托利细胞/超微结构睾丸/病理学紧密连接部/代谢紧密连接部/超微结构动物男(雄)性小鼠大鼠

来源：评论

学校读者我要写书评

暂无评论

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

请选择保存的检索档案：

请选择收藏分类：

通借通还

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：