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1.
Human mitochondrial tRNAs in health and disease 总被引:6,自引:0,他引:6
Florentz C Sohm B Tryoen-Tóth P Pütz J Sissler M 《Cellular and molecular life sciences : CMLS》2003,60(7):1356-1375
The human mitochondrial genome encodes 13 proteins, all subunits of the respiratory chain
complexes and thus involved in energy metabolism. These genes are translated by 22 transfer RNAs
(tRNAs), also encoded by the mitochondrial genome, which form the minimal set required for reading
all codons. Human mitochondrial tRNAs gained interest with the rapid discovery of correlations
between point mutations in their genes and various neuromuscular and neurodegenerative disorders.
In this review, emerging fundamental knowledge on the structure/function relationships of these
particular tRNAs and an overview of the large variety of mechanisms within translation, affected by
mutations, are summarized. Also, initial results on wide-ranging molecular consequences of mutations
outside the frame of mitochondrial translation are highlighted. While knowledge of mitochondrial
tRNAs in both health and disease increases, deciphering the intricate network of events leading
different genotypes to the variety of phenotypes requires further investigation using adapted
model systems.Received 3 December 2002; received after revision 14 January 2003; accepted 27 January 2003 相似文献
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Malaguarnera L 《Cellular and molecular life sciences : CMLS》2006,63(24):3018-3029
The enzyme chitotriosidase (ChT), the human analogue of chitinases from non-vertebrate species, is one of the most abundant
and indicative proteins secreted by activated macrophages. Its enzymatic activity is elevated in serum of patients suffering
from Gaucher’s disease type 1 and in some other inherited lysosomal storage disorders, as well as in diseases in which macrophages
are activated. The last decade has witnessed the appearance of a substantial number of studies attempting to unravel its cellular
functions, which have yet not been fully defined. A great deal of progress has been made in the study of the physiological
roles of ChT. This review is looks at the key areas of investigations addressed to further illuminate whether ChT activation
might have different functional meanings in various diseases.
Received 7 June 2006; received after revision 24 July 2006; accepted 21 September 2006 相似文献
4.
From Creutzfeldt-Jakob disease (CJD) to variant CJD through Gerstmann-Str?ussler-Scheinker syndrome, kuru and fatal familial insomnia, the journey leading to current understanding of the basic aspects of human prion diseases has been full of unexpected, but often dramatic and always fascinating twists. Recent progress in modeling prion diseases and characterization of the various prion protein forms reveal that such a wide spectrum of the diseases is associated with the chameleon-like conformational features of prions. 相似文献
5.
Hongyu Hu 《科学通报(英文版)》2001,46(1):1-3
Abnormal protein aggregation or amyloid is the major cause of many neurodegenerative disorders. The present review focuses on the correlation between sequence and structure features of proteins related to the diseases and abnormal protein aggregation. Recent progress has improved our knowledge on understanding the mechanism of amyloid formation. We suggest a nucleation model for ordered protein aggregation, which can also explain pathogenesis mechanisms of these neurodegenerative diseases in vivo. 相似文献
6.
60-year prion and scrapie research has led to a dilemma in understanding the unknown aetiology of the infectious neurodegenerative
disorders with intriguing features. Current progress and dilemma in prion research are briefly but critically reviewed. Instead
of providing a comprehensive coverage of the research history, attentions in this view are drawn toward both the major breakthrough
in the advancement of protein-only hypothesis, and the puzzle why this hypothesis has not been fully accepted. In order to
resolve the prion enigma in neuroscience, it is suggested that both technical and concept barriers remain to be crossed. Since
prion research is a multi-interdisciplinary subject, this view is intended to both facilitate a better understanding of prion
phenomenon by more scientists in natural science, and invite scientists outside the fields of molecular genetics and protein
science for collaboration. 相似文献
7.
60-year prion and scrapie research has led to a dilemma in understanding the unknown aetiology of the infectious neurodegenerative disorders with intriguing features. Current progress and dilemma in prion research are briefly but critically reviewed. Instead of providing a comprehensive coverage of the research history, attentions in this view are drawn toward both the major breakthrough in the advancement of protein-only hypothesis, and the puzzle why this hypothesis has not been fully accepted. In order to resolve the prion enigma in neuroscience, it is suggested that both technical and concept barriers remain to be crossed. Since prion research is a multi-interdisciplinary subject, this view is intended to both facilitate a better understanding of prion phenomenon by more scientists in natural science, and invite scientists outside the fields of molecular genetics and protein science for collaboration. 相似文献
8.
Melatonin regulation of antioxidant enzyme gene expression 总被引:15,自引:0,他引:15
Mayo JC Sainz RM Antoli I Herrera F Martin V Rodriguez C 《Cellular and molecular life sciences : CMLS》2002,59(10):1706-1713
Antioxidant enzymes (AOEs) are part of the primary cellular defense against free radicals induced by toxins and/or spontaneously
formed in cells. Melatonin (MLT) has received much attention in recent years due to its direct free radical scavenging and
antioxidant properties. In the present work we report that MLT, at physiological serum concentrations (≈ 1 nM), increases
the mRNA of both superoxide dismutases (SODs) and glutathione peroxidase (GPx) in two neuronal cell lines. The MLT effect
on both SODs and GPx mRNA was mediated by a de novo synthesized protein. MLT alters mRNA stability for Cu-Zn SOD and GPx.
Experiments with a short time treatment (pulse action) of MLT suggest that the regulation of AOE gene expression is likely
to be receptor mediated, because 1-h treatment with MLT results in the same response as a 24-h treatment.
Received 18 June 2002; received after revision 5 August 2002; accepted 27 August 2002
RID="*"
ID="*"Corresponding author. 相似文献
9.
阐述了绿茶多酚的化学组成、生物代谢以及在药物开发领域的进展,主要集中在对癌症、神经退行性疾病、糖尿病的预防和治疗方面.茶多酚潜在的抗肿瘤活性可以通过干预肿瘤发生、发展、转移等各个阶段来进行,但茶多酚发展成为治疗药物还是面临很多挑战,比如,低生物药效率.基于纳米颗粒的药物传递系统相比单纯的茶多酚具有特别的优势,同时,为了处理新出现的安全问题和潜在的局部药物过量效应,有必要确定包裹纳米粒子以后的药物实际用量和药理学机制. 相似文献
10.
干细胞移植是目前治疗器官损伤、神经退行性疾病研究的热点,国内外学者分别对神经干细胞、胚胎干细胞等进行了探索,渴望从中找出细胞移植领域的候选细胞,但是这些细胞大多面临着伦理、来源、免疫排斥等多方面的问题.然而,近年来发现的骨髓间充质干细胞在一定程度上弥补了这一不足.骨髓间充质干细胞凭借其多向分化潜能、强大的自我复制能力和可移植性,成为细胞移植、基因治疗中重要的候选细胞.骨髓间充质干细胞移植为器官损伤、神经退行性疾病的临床治疗带来了新的希望,本文对其生物学特性及临床应用方面做一综述. 相似文献