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Introduction A hexanucleotide (GGGGCC) repeat expansion in the first intron of the C9orf72 gene was recently identified as a major contributing factor to the chromosome 9p21-linked diseases amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (). As previously reported, the C9orf72 mutation accounts for 23.5–47% of familial ALS/FTD and 4.1–21.0% of sporadic ALS in white populations (;; ). The pathogenic mechanism of repeat expansions primarily includes interference with the normal expression of the encoded protein or the loss of protein function through the generation of abnormal toxic RNA foci that disrupt normal cellular pathways (; ). Essential John Waite Jan 14, 1992. By John Waite. $2.52 (9 used & new offers) Audio Cassette. $11.99 $ 11 99. Only 1 left in stock - order soon. More Buying Choices. $7.50 (4 used & new offers) 3.5 out of 5 stars 8. Temple Bar Apr 11, 1995. By John Waite. $25.25 $ 25 25 Prime. FREE Shipping on eligible orders. Essential John Waite. 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First, in the examination of clinical phenotypes, relatives of patients with ALS have an increased risk for developing PD and AD, additionally, some ALS/FTD patients have developed the associated features of Parkinsonism and movement disorders (;; ). Second, the presence of TAR DNA-binding protein-43(+) intranuclear inclusions, which are the pathological feature of chromosome 9p21-linked ALS/FTD, have been detected in PD and AD patients (; ). Finally, mutations in the microtubule-associated protein tau (MAPT) gene could cause a spectrum of phenotypes which include ALS, Parkinsonism, and cognitive impairment (). Given the considerations above, one question has yet to be addressed. Could the C9orf72 repeat expansions account for other neurodegenerative disorders, such as AD, PD, and essential tremor (ET)? Download lagu buat organ format midi. Yuk download koleksi song midi terbaru di tahun 2017 ini, dan apa-apa saja lagu midi keyboard yang telah disediakan untuk para sobat keyboa. Download Midi Lagu Midi Karaoke Midi Terbaru Midi Dangdut Inimidiku Midi full lirik Midi untuk Karaoke style organt Midi Style Midi Pop. § Software Buat Midi. ![]() In C9orf72, Repeat expansions exceeding 30 units have been suggested to be pathological in ALS/FTD patients (). Interestingly, pathogenic expansions have also been observed in patients with PD, AD, progressive supranuclear palsy, corticobasal degeneration, and Lewy body dementia (;; ), which further indicates that the phenotypes that are associated with repeat expansions could include the spectrum of cognitive impairment and movement disorder syndromes. In addition, a previous study has demonstrated that the role of intermediate repeats (7–24 repeat units) is strongly associated with these diseases and the expression of C9orf72. The significantly decreased transcriptional activity of C9orf72 with an increasing number of normal repeats indicates that intermediate repeats may act as predisposing alleles and favors the loss-of-function disease mechanism (). In this study, we first assess the prevalence of C9orf72 repeat expansions in a large cohort of Chinese Han patients with AD, PD, or ET to determine whether repeat expansions play a role in these three common disorders. Furthermore, we explore whether repeat expansions of intermediate repeats might be a risk factor for AD, PD, or ET, and/or could affect the age at onset in patients with these three diseases. Materials and Methods Study Samples Three independent series of patients participated in this study: the first cohort of 911 sporadic PD patients that met the UK brain bank diagnosis criteria (); the second cohort of 279 sporadic AD patients that met the NINCDS-ADRDA criteria for probable or definite AD (); and the third cohort of 152 ET patients that met the Washington Heights-Inwood Genetic Study of ET (WHIGET) diagnosis criteria (). All patients were recruited from the outpatient neurology clinics of the Xiangya Hospital, Central South University. In total, 314 healthy Chinese individuals were recruited from the Xiangya Wellness Center as a control group. Informed consents for participation in the study were obtained from all subjects, including patients and controls. This study received prior approval by the institutional review board and the ethics committee of the Xiangya Hospital, Central South University. 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Март 2019
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