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Substitution of cysteine for glycine-α1-691 in the proα1(I) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution

机译：在Proα1（i）中甘氨酸-α1-691中的半胱氨酸替代I型α1（i）链中含有致命骨肉成骨的催化剂的链条的链条稳定地破坏了位点C末端的三重螺旋到取代

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B Steinmann; A Westerhausen; C D Constantinou; A Superti-Furga; D J Prockop;
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1. Substitution of cysteine for glycine-α1-691 in the proα1(I) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution [J] . A Superti-Furga, A Westerhausen, B Steinmann, The biochemical journal . 1991,第3期

机译：在具有致死性成骨不全的先证者中，I型前胶原的proα1（I）链中的半胱氨酸被甘氨酸-α1-691取代，从而使取代位置C端的三重螺旋不稳定
2. Two cysteine substitutions in procollagen I: a glycine replacement near the N-terminus of α1(I) chain causes lethal osteogenesis imperfecta and a glycine replacement in the α2(I) chain markedly destabilizes the triple helix [J] . A Fertala, A Westerhausen, D J Prockop, The biochemical journal . 1993,第1期

机译：前胶原I中的两个半胱氨酸替代：α1（I）链N端附近的甘氨酸替代导致致死性成骨不全;α2（I）链中的甘氨酸替代显着破坏三重螺旋的稳定性
3. SUBSTITUTIONS OF ASPARTIC ACID FOR GLYCINE-220 AND OF ARGININE FOR GLYCINE-664 IN THE TRIPLE HELIX OF THE PRO-ALPHA-1(I) CHAIN OF TYPE I PROCOLLAGEN PRODUCE LETHAL OSTEOGENESIS IMPERFECTA AND DISRUPT THE ABILITY OF COLLAGEN FIBRILS TO INCORPORATE CRY [J] . Culbert AA., Atkinson M., Byers PH., The Biochemical Journal . 1995,第Pta3期

机译：I型胶原蛋白原pro-α-1（I）链的三重螺旋中的丙二酸-220精氨酸和精氨酸-664的精氨酸替代产生了致命的成骨性骨生成不良，破坏了胶原蛋白纤维的能力。
4. A-site Substitution Effect of Perovskite-type Cobalt and Manganese Oxides on Two-step Water Splitting Reaction for Solar Hydrogen Production [C] . Hiroshi Kaneko, Takumi Hasegawa, Kohei Mori International Conference on Concentrating Solar Power and Chemical Energy Systems . 2017

机译：钙钛矿型钴和锰氧化物对太阳能氢气生产两步水分裂反应的现场替代作用
5. Optimization of Li-ion Conductivity of Garnet-type Li 5La3Nb2O12 by Nb-site Substitution Approach. [D] . Pinzaru, Dana Irina. 2015

机译：Nb-位取代法优化石榴石型Li 5La3Nb2O12锂离子电导率
6. Substitution of cysteine for glycine-alpha 1-691 in the pro alpha 1(I) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution. [O] . B Steinmann, A Westerhausen, C D Constantinou, 1991

机译：在具有致死性成骨不全的先证者中I型胶原蛋白的pro alpha 1（I）链中的半胱氨酸替代甘氨酸-alpha 1-691会破坏取代基C端的三重螺旋的稳定性。
7. A lethal variant of osteogenesis imperfecta has a single base mutation that substitutes cysteine for glycine 904 of the alpha 1(I) chain of type I procollagen. The asymptomatic mother has an unidentified mutation producing an overmodified and unstable type I procollagen. [O] . Constantinou, C D, Nielsen, K B, Prockop, D J 1989

机译：成骨不全症的致死变体具有一个单碱基突变，该突变将半胱氨酸替换为I型胶原原α1（I）链的甘氨酸904。无症状的母亲有一个无法识别的突变，产生了过度修饰和不稳定的I型胶原蛋白。

Substitution of cysteine for glycine-α1-691 in the proα1(I) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution

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