关键词：
Adult-onset type II citrullinemia (CTLN2);Aspartate-glutamate carrier (AGC);Citrin;Malate-aspartate shuttle;Neonatal intrahepatic cholestatic hepatitis (NICCD);Retrotransposal insertion;SLC25A13
摘要：
Deficiency of citrin, liver-type mitochondrial aspartate-glutamate carrier, is an autosomal recessive disorder caused by mutations of the SLC25A13 gene on chromosome 7q21.3 and has two phenotypes: neonatal intrahepatic cholestatic hepatitis (NICCD) and adult-onset type II citrullinemia (CTLN2). So far, we have described 19 SLC25A13 mutations. Here, we report 13 novel SLC25A13 mutations (one insertion, two deletion, three splice site, two nonsense, and five missense) in patients with citrin deficiency from Japan, Israel, UK, and Czech Republic. Only R360X was detected in both Japanese and Caucasian. IVS16ins3kb identified in a Japanese CTLN2 family seems to be a retrotransposal insertion, as the inserted sequence (2,667-nt) showed an antisense strand of processed complementary DNA (cDNA) from a gene on chromosome 6 (C6orf68), and the repetitive sequence (17-nt) derived from SLC25A13 was found at both ends of the insert. All together, 30 different mutations found in 334 Japanese, 47 Chinese, 11 Korean, four Vietnamese and seven non-East Asian families have been summarized. In Japan, IVS16ins3kb was relatively frequent in 22 families, in addition to known mutations IVS11 + 1G > A, 851del4, IVS13 + 1G > A, and S225X in 189, 173, 48 and 30 families, respectively; 851del4 and IVS16ins3kb were found in all East Asian patients tested, suggesting that these mutations may have occurred very early in some area of East Asia.
期刊：
JOURNAL OF PHYSICAL CHEMISTRY A,2008年112(2):305-311 ISSN：1089-5639
通讯作者：
Xiao, Xiaoming
作者机构：
[Rong, Chunying; Xiao, Xiaoming; Liu, Shubin; Huang, Ying] Hunan Normal Univ, Coll Chem & Chem Engn, Changsha 410081, Hunan, Peoples R China.;[Huang, Ying] Hunan Univ Tradit Chinese Med, Sch Pharm, Changsha 410007, Hunan, Peoples R China.;[Zhong, Aiguo] Taizhou Coll, Dept Chem, Linhai 317000, Zhejiang, Peoples R China.;[Liu, Shubin] Univ N Carolina, Renaissance Comp Inst, Chapel Hill, NC 27599 USA.
通讯机构：
[Xiao, Xiaoming] H;Hunan Normal Univ, Coll Chem & Chem Engn, Changsha 410081, Hunan, Peoples R China.
摘要：
Porphyrin and pincer complexes are both important categories of compounds in biological and catalytic systems. The idea to combine them is computationally investigated in this work. By employment of density functional theory (DFT), conceptual DFT, and time-dependent DFT approaches, structure, spectroscopy, and reactivity properties of porphyrin pincers are systematically studied for a selection of divalent metal ions. We found that the porphyrin pincers are structurally and spectroscopically different from their precursors and are more reactive in electrophilic and nucleophilic reactions. A few quantitative linear/exponential relationships have been discovered between bonding interactions, charge distributions, and DFT chemical reactivity indices. These results are implicative in chemical modification of hemoproteins and understanding chemical reactivity in heme-containing and other biologically important complexes and cofactors.
作者机构：
[Liu, Hao-Ran; Zeng, Chun-Jiao; Liu, Ying-Lin; Li, Yan-Lai] Hunan Univ, Dept Pharmacol, Coll Chem & Chem Engn, Changsha 410082, Hunan, Peoples R China.;[He, Hai-Bo] Zhejiang Univ, Inst Chinese Herb Med, Coll Pharmaceut Sci, Hangzhou 310058, Zhejiang, Peoples R China.;[Peng, Xiang-Dong] Cent S Univ, Xiangya Hosp 3, Dept Pharm, Changsha, Hunan, Peoples R China.;[He, Gui-Xia] Hunan Univ TCM, Coll Pharm, Dept Nat Pharmaceut Chem, Changsha, Hunan, Peoples R China.
通讯机构：
[He, Hai-Bo] Z;Zhejiang Univ, Inst Chinese Herb Med, Coll Pharmaceut Sci, Hangzhou 310058, Zhejiang, Peoples R China.
摘要：
Total saponin of Solanum lyratum Thunb (TSSLT), a species of natural biologically active substances isolated from Solanum lyratum Thunb, possesses various bioactivities. It has been proposed that the induction of apoptosis may be the basis of its antitumor activity. However, the molecular mechanism underlying the total saponin-induced apoptotic process remains unknown. In the present study, we describe the anti-proliferative effect of TSSLT on human cervical cancer cells (Hela). The TSSLT induced apoptosis of Hela in a time-dependent manner with an IC50 for cell viability of 6 μg/ml. The TSSLT-induced cell death was characterized by changes in cell morphology, DNA fragmentation, activation of caspase-like activities, poly (ADP-ribose) polymerase (PARP) cleavage and release of cytochrome c (cyt c) into cytosol. TSSLT activated various caspases such as caspase-3, -8, and -9 (like) activities but not caspase-1 like activity. The cell death was completely prevented by the pan caspase inhibitor benzyloxy carbonyl-Val-Ala-Asp- fluoromethyl-ketone (Z-VAD-FMK). More than 80% cell survival was observed in the presence of a caspase-3 inhibitor. In addition, treatment with TSSLT induced the increase of Bax:Bcl-2 ratio in Hela cells. These results suggest that the induction of apoptosis by TSSLT involves multiple pathways antigen including death receptor and mitochondrial pathway and strongly suggest that the mitochondrial pathway was mediated by low expression of Bcl-2 and upregulation of Bax, release of cyt c and subsequent activation of caspase-3 followed by down stream events leading to apoptotic cell death.
摘要：
A dye intermediate, 1-amino-8-naphthol-3,6-disulfonic acid sodium (ANDS) was first used to selectively recognize Hg(II) in aqueous solutions with its fluorescence being strong quenched. The fluorescence quenching of ANDS was attributed to the formation of an inclusion complex between Hg(II) and ANDS by 2:1 complex ratio (K= 6.2 x 10(9)), which has been utilized as the basis of the fabrication of the Hg(II)-sensitive fluorescent chemosensor. The analytical performance characteristics of the proposed chemosensor were investigated. The sensor shows a linear response toward Hg(II) in the concentration range 2.9 x 10(-6) to 5.5 x 10(-5) M with a limit of detection of 5.3 x 10(-7) M, and a working pH range from 5.0 to 9.0. It shows excellent selectivity for Hg(II) over a large number of cations such as alkali, alkaline earth and transitional metal ions. The proposed method was utilized successfully for the detection of Hg2+ in water samples. (C) 2007 Elsevier B.V. All rights reserved.
作者机构：
[曹俊岭] Institute of Chinese Medicine, 302 Hospital of PLA, Beijing 100039, China. cj10506@sina.com
关键词：
人参皂苷;含量测定
摘要：
目的:为8种人参皂苷的含量测定建立一套方法.方法:色谱柱:ODS柱(Kromasil 250mm×4.6mm,5μm),DAD检测器;流动相:A为乙腈,B为水,梯度洗脱0～35 min 19%A,35～55 min 19%～29%A,55～75 min29%A,75～100 min 29%～40%A.流速:1 ml/min,温度:室温,检测波长:203 nm.结果:用本方法对各种皂苷的测定结果:稳定性试验RSD为0.55%～2.26%,精密度试验RSD为0.85%～1.93%,重复性试验RSD为0.97%～2.72%.结论:本法稳定性好,精确度高,重复性好,可作为测定这8种皂苷的一个较好的方法.
作者机构：
[鲁耀邦; 彭菲] Department of Biotechnology, Pharmacy College, Hunan University of Traditional Chinese Medicine, Changsha, Hunan, 410007, China;[李孟贤; Kobayashi K.; Saheki T.] Department of Molecular Metabolism and Biochemical Genetics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, 890-8544, Japan
通讯机构：
[Lu, Y.-B.] D;Department of Biotechnology, Pharmacy College, Hunan University of Traditional Chinese Medicine, China
作者机构：
College of Pharmacy, Hunan College of Traditional Chinese Medicine, Changsha 410007, China;First Hospital Attached to Hunan College of Traditional Chinese Medicine, Changsha 410007, China;[郭建生] College of Pharmacy, Hunan College of Traditional Chinese medicine, Changsha 410007, China
通讯机构：
College of Pharmacy, Hunan College of Traditional Chinese Medicine, China
