作者: 投票 時(shí)間: 2025-3-22 00:04
Advances in Experimental Medicine and Biologyhttp://image.papertrans.cn/i/image/466465.jpg作者: 神圣不可 時(shí)間: 2025-3-22 04:06 作者: Intentional 時(shí)間: 2025-3-22 05:06 作者: hidebound 時(shí)間: 2025-3-22 09:45
978-94-007-3056-4The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature B.V. 200作者: confide 時(shí)間: 2025-3-22 14:15 作者: Creditee 時(shí)間: 2025-3-22 19:40 作者: Jocose 時(shí)間: 2025-3-22 22:24
Standards of Care for Duchenne Muscular Dystrophy: Brief Treat-NMD Recommendations,d on the generation of brief standards of care for DMD, which are presented here and are available via the TREAT-NMD website (.). Guidelines are presented for diagnostics, neurological follow up, gastrointestinal and nutritional issues, respiratory and cardiac care as well as orthopaedics, rehabilitation, psychosocial interventions and oral care.作者: fulmination 時(shí)間: 2025-3-23 02:50
Phenotype and Clinical Evolution of Charcot-Marie-Tooth Disease Type 1A Duplication,d early childhood; (iii) proximal lower-limb musculature involvement as a late phenotypic feature; and (iv) minimal adult phenotype. We also briefly revise genetic, electrophysiological, pathological and neuroimaging data of the disease.作者: absolve 時(shí)間: 2025-3-23 06:23
Pathology and Diagnosis of Muscular Dystrophies,approach, necessary to conduct genetic studies and to offer an appropriate genetic counseling. The application of molecular medicine in genetic muscular dystrophies also brings great expectations to the therapeutic management of these patients.作者: Restenosis 時(shí)間: 2025-3-23 15:37 作者: 使聲音降低 時(shí)間: 2025-3-23 18:08
Biology of Peripheral Inherited Neuropathies: Schwann Cell Axonal Interactions,ll be presented. Understanding the molecular basis of Schwann cell-axonal interactions will not only increase the understanding of PNS biology but also identify therapeutic targets for inherited neuropathies.作者: 共同生活 時(shí)間: 2025-3-24 02:05 作者: SMART 時(shí)間: 2025-3-24 05:30
rikas besch?ftigten. Lesenswerte und engagierte Aufarbeitungen wie z.B. Christoph Marx’ (2012) Buch zur ?Geschichte und Gegenwart“, Renate Wilke-Launers (2010) ?Katerstimmung am Kap“ oder auch der in Zusammenarbeit mit südafrikanischen sozialen Bewegungen entstandene Sammelband ?Grenzen der Freiheit作者: Truculent 時(shí)間: 2025-3-24 10:31 作者: 擔(dān)心 時(shí)間: 2025-3-24 14:32 作者: 豪華 時(shí)間: 2025-3-24 17:09
Samuel Ignacio Pascual Pascualanach einen deutlichen Wachstumskurs eingeschlagen. Teilweise erreichte die j?hrliche Steigerung des Bruttoinlandsprodukts (BIP) über 5%. Im Jahre 1999 war das BIP wieder auf dem Stand von 1989. In der letzten Zeit hat sich das j?hrliche Wachstum jedoch abgeschw?cht. In 2003 lag es bei vergleichswei作者: colostrum 時(shí)間: 2025-3-24 22:36
Julio Montoya,Ester López-Gallardo,María Dolores Herrero-Martín,í?igo Martínez-Romero,Aurora Gómez-DEntwicklung des Parteiensystems und die Struktur der politischen Auseinandersetzungen. Er zeigt, dass sich unmittelbar nach dem Systemwechsel ein stabiles, bipolares Parteiensystem entwickelte, in dem fünf Parteien kontinuierlich parlamentarisch vertreten waren. Bis heute pr?gen zwei tradierte Haupt作者: 惰性氣體 時(shí)間: 2025-3-24 23:30
Rafael Artuch,Leonardo Salviati,Sandra Jackson,Michio Hirano,Plácido Navasxperten versucht wird, das Werk aus einem Guss geraten zu lassen wie am Anfang der Weimarer Republik durch die Vorarbeiten von Hugo Preu?, sind komplizierte Verhandlungen n?tig, um Kompromisse zu erzielen. Die Entstehung der Bundesrepublik war im Gegensatz zu früheren verfassunggebenden Prozessen an作者: obsession 時(shí)間: 2025-3-25 05:55
Pathology and Diagnosis of Muscular Dystrophies, classified into autosomal recessive and dominant forms, the former being by far the commonest. Genetic analyses have led to the description of 14 recessive forms and 7 dominant. In four of the dominant forms the chromosomal loci have been localised but the gene and the protein have not been yet ide作者: 廣告 時(shí)間: 2025-3-25 08:23
Standards of Care for Duchenne Muscular Dystrophy: Brief Treat-NMD Recommendations,improve outcomes in DMD but these are not uniformly applied. This leads to inequality in access to treatment, as well as problems for planning controlled trials of future therapeutics. A worldwide effort is underway to generate care guidelines for DMD, which involves the Centre for Disease Control i作者: infringe 時(shí)間: 2025-3-25 12:15 作者: BILL 時(shí)間: 2025-3-25 17:44
,Phenotype Variations in Early Onset Pompe Disease: Diagnosis and Treatment Results with Myozyme?,cogen accumulation in the lysosomes, leading to lysosomal swelling, cellular damage and organ dysfunction. Patient age at the onset of Pompe disease symptoms and the rate of deterioration can vary considerably..In early onset patients (the classical infantile form) this glycogen accumulation leads t作者: 緯度 時(shí)間: 2025-3-25 21:55 作者: Anthropoid 時(shí)間: 2025-3-26 04:11 作者: 文藝 時(shí)間: 2025-3-26 07:51 作者: Vertical 時(shí)間: 2025-3-26 11:29
Coenzyme Q10 Deficiencies in Neuromuscular Diseases,ransition pore and uncoupling proteins. Furthermore, this compound is a specific substrate for enzymes of the fatty acids β–oxidation pathway and pyrimidine nucleotide biosynthesis. Furthermore, CoQ is an antioxidant that acts in all cellular membranes and lipoproteins. A complex of at least ten nuc作者: 凹處 時(shí)間: 2025-3-26 14:56
The Role of Mitochondrial Network Dynamics in the Pathogenesis of Charcot-Marie-Tooth Disease,ary defect of either the mtDNA or nuclear genome encoded subunits of the respiratory chain. These organelles have also been directly involved in the pathogenesis of Mendelian neurodegenerative disorders caused by mutations in nuclear-encoded proteins targeted to mitochondria, such as Friedreich atax作者: 密切關(guān)系 時(shí)間: 2025-3-26 18:51
Pathogenesis and Treatment of Mitochondrial Disorders,efects of the mitochondrial respiratory chain (RC), has made great strides. Mitochondrial diseases can be due to mutations in mitochondrial DNA (mtDNA) or in nuclear DNA (nDNA) and each group can be subdivided into more specific classes. Thus, mtDNA-related disorders can result from mutations in gen作者: emulsify 時(shí)間: 2025-3-26 22:31 作者: arousal 時(shí)間: 2025-3-27 03:24 作者: 擋泥板 時(shí)間: 2025-3-27 05:42
Genotypes & Sensory Phenotypes in 2 New X-Linked Neuropathies (CMTX3 and dSMAX) and Dominant CMT/HMvely easy in single patients but subtle sensory findings can vary in different affected individuals in a family. We examined the extent of sensory involvement in different individuals in two new X-linked neuropathy syndromes (CMTX3 and dSMAX) and in some dominantly inherited mainly motor neuropathie作者: 肉體 時(shí)間: 2025-3-27 12:22
Natural History and Treatment of Peripheral Inherited Neuropathies,ation type, but considerable phenotypic variability may occur also for the same CMT type. Research is focused on possible modifier factors particularly in CMT1A associated with Peripheral Myelin Protein 22 (PMP22) overexpression. Natural history studies are important to define disease course in diff作者: Scintillations 時(shí)間: 2025-3-27 17:05
Spinal Muscular Atrophy During Human Development: Where Are the Early Pathogenic Findings?,ne 1 (SMN1). The SMN2 gene, which is the highly homologous SMN1 copy that is present in all patients, is unable to prevent the disease. SMA patients can be classified into four groups based on age at onset and acquired milestones (type I or severe acute disease, with onset before 6 months; type II, 作者: mendacity 時(shí)間: 2025-3-27 20:23
Spinal Muscular Atrophy,ations of the survival of motor neuron gene (.) leading to a reduction of the SMN protein amount. The identification of SMN interacting proteins involved in the formation of the spliceosome and splicing changes in SMN-deficient tissues of mutant mice strongly support the view that SMN is involved in作者: Left-Atrium 時(shí)間: 2025-3-27 22:17
Friedreich Ataxia: An Update on Animal Models, Frataxin Function and Therapies, heart. Although FRDA is considered a rare disease, is the most common inherited ataxia. It is caused by loss-of-function mutations in the . gene, mainly an expanded GAA triplet repeat in the intron 1. The genetic defect results in the reduction of frataxin levels, a protein targeted to the mitochon作者: 難管 時(shí)間: 2025-3-28 04:08
Genetics and Pathogenesis of Inherited Ataxias and Spastic Paraplegias,toms and also, with a remarkable number of involved loci/genes. Inherited ataxias are clinically characterized by progressive degeneration of cerebellum and spinocerebellar tracts of the spinal cord associated with a variable combination of signs of central and peripheral nervous system. Hereditary 作者: Provenance 時(shí)間: 2025-3-28 09:20 作者: 暫時(shí)休息 時(shí)間: 2025-3-28 14:12
,Phenotype Variations in Early Onset Pompe Disease: Diagnosis and Treatment Results with Myozyme?,A in dried blood sample followed by GAA assessment in lymphocytes or fibroblasts or by the genetic study of mutations..Besides non specific multiprofesional management, since 2006 there is a specific enzyme replacement therapy (ERT), Myozyme.), which compensates for the missing enzyme by i.v. admini作者: micronized 時(shí)間: 2025-3-28 16:03 作者: PAC 時(shí)間: 2025-3-28 21:20 作者: foreign 時(shí)間: 2025-3-29 02:19
Pathogenesis and Treatment of Mitochondrial Disorders,he synthesis and composition of mitochondrial membrane phospholipids; and (vi) encode proteins involved in mitochondrial dynamics..In contrast to this wealth of knowledge about etiology, our understanding of pathogenic mechanism is very limited. We discuss pathogenic factors that can influence clini作者: 愚笨 時(shí)間: 2025-3-29 06:57 作者: Nebulizer 時(shí)間: 2025-3-29 10:54 作者: 小爭(zhēng)吵 時(shí)間: 2025-3-29 14:00 作者: 自負(fù)的人 時(shí)間: 2025-3-29 17:01
Spinal Muscular Atrophy,of the degenerative process in SMA and the identification of the defective molecular pathway downstream from the SMN defect will provide further exciting insight into this disease in the near future. They should contribute to clarify the pathophysiology of SMA, the function of SMN and should help in作者: 放肆的我 時(shí)間: 2025-3-29 21:05 作者: 抓住他投降 時(shí)間: 2025-3-30 01:47
Cristina Ugalde,María Morán,Alberto Blázquez,Joaquín Arenas,Miguel A. Martín作者: 流浪 時(shí)間: 2025-3-30 04:22 作者: OUTRE 時(shí)間: 2025-3-30 08:12 作者: 用不完 時(shí)間: 2025-3-30 13:25 作者: DIS 時(shí)間: 2025-3-30 19:17
Jérémie Vitte,Ruben Attali,Nasim Warwar,Irena Gurt,Judith Melki作者: 優(yōu)雅 時(shí)間: 2025-3-30 21:43 作者: 不溶解 時(shí)間: 2025-3-31 03:44
Jahren (1999). Ende 2013 starb Mandela und es folgte das erste Jahr für Südafrika ohne ., den ?Vater der Nation“. Im Mai 2014 wurden zudem zum fünften Mal – friedlich und frei – Parlaments- und Kommunalwahlen abgehalten, die zu spannenden Ver?nderungen führten (s.u.). All diese Ereignisse regten zu 作者: BANAL 時(shí)間: 2025-3-31 07:35
Carmen Navarro,Susana Teijeira,Beatriz San Millánossen worden waren, einige von ihnen mehrmals (Marinovich 2012). Zehn weitere Menschen, einschlie?lich Polizisten und privates Sicherheitspersonal, waren bereits in der vorhergegangenen Woche get?tet worden. Die Polizei nahm zudem hunderte der überlebenden fest. Viele berichteten, dass sie im Gewahr作者: forebear 時(shí)間: 2025-3-31 11:32
Bjarne Uddgen vornehmlich auf die gestiegene Exportnachfrage stützen k?nnen, w?hrend die Einkommen im wesentlichen stagnieren und ?ffentliche Investitionen mangels Finanzierbarkeit zurückgehen werden (vgl. Bundesagentur 2003: 6 ff). Erst für das Jahr 2005 sieht die Prognose eine günstigere Entwicklung voraus;作者: indemnify 時(shí)間: 2025-3-31 13:28 作者: peak-flow 時(shí)間: 2025-3-31 20:50
Julio Montoya,Ester López-Gallardo,María Dolores Herrero-Martín,í?igo Martínez-Romero,Aurora Gómez-D Mittelklasse zu ersetzen und war seit 2010 mit seiner spezifischen strategischen Ausrichtung sehr erfolgreich, w?hrend das links-liberale Lager sich zunehmend heterogenisierte. Der Beitrag zeigt au?erdem, mit welchen Ma?nahmen Fidesz-KDNP seinen Einfluss abstützte.作者: 網(wǎng)絡(luò)添麻煩 時(shí)間: 2025-3-31 22:35 作者: 物質(zhì) 時(shí)間: 2025-4-1 04:17 作者: 周興旺 時(shí)間: 2025-4-1 07:56 作者: CARK 時(shí)間: 2025-4-1 14:14
Measuring the?Effects of?Confidants on?Privacy in?Smart Contractsility or availability..In this paper, we introduce measures for assessing the impact of confidants for decisions within smart contracts on privacy. We model smart contracts in form of inter-organizational business processes and provide modeling constructs for privity requirements and the inclusion o作者: Malcontent 時(shí)間: 2025-4-1 17:04
Changing Political Culture in Eastern Europe,adges with a picture of Gorbachev and revived an old GDR slogan: To learn from the Soviet Union — means learning victory’. This time it was used, however, in an ironic way. The GDR was kept by her own slogans because it was unable to outlaw the use of the badges.作者: 格言 時(shí)間: 2025-4-1 19:35
Julian Keil,Frank Dickmann,Lars Kuchinkeen Beimengungen enthaltene Sauerstoff oxydiert die Elektroden und erschwert reproduzierbare Messungen sehr. Um den sch?dlichen Einflu? des Sauerstoffs zu vermindern, haben wir dem technischen Stickstoff etwas Wasserstoff zugesetzt, der die Elektroden von Oxyden wieder befreit.作者: Acetabulum 時(shí)間: 2025-4-2 00:00
ray detecting camera systems and computer links enable the clinical investigator to image different regions of the body and to quantify organ function. Parallel developments have also occurred in the field of radiopharmaceuticals, and today a wide range of products is available that will exhibit spe作者: 好忠告人 時(shí)間: 2025-4-2 03:23
