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Research progress of cystic fibrosis and its therapies
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Lin LIN1, Yan WANG1, Chuan-bin WU2, Chun-e ZHU1, *
Acta Pharmaceutica Sinica | 2022, 57(7) : 2024 - 2031
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Acta Pharmaceutica Sinica | 2022, 57(7): 2024-2031
• Reviews •
Research progress of cystic fibrosis and its therapies
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Lin LIN1, Yan WANG1, Chuan-bin WU2, Chun-e ZHU1, *
Affiliations
  • 1. School of Chinese Materia Medica, Guangdong Pharmaceutical University, Guangzhou 510006, China
  • 2. School of Pharmaceutical Sciences, Jinan University, Guangzhou 510032, China
Published: 2022-07-12 doi: 10.16438/j.0513-4870.2021-1750
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Cystic fibrosis (CF) is a common and life-threatening autosomal recessive disorder in Caucasians populations. The disease is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The absence of mature CFTR at the correct cellular location or dysfunction of CFTR proteins has been observed in CF patients. CF is frequently accompanied by a variety of complex complications, such as impairments in pulmonary functions, which may lead to successive infections and respiratory failure. Recently, with the understanding of the pathogenesis of CF, a wide array of therapeutic strategies for the treatment of CF has been designed. This review summarizes pathogenic mechanisms of CF, mechanisms of action of drugs, routes of administration, and new drug development as well as provides insights into the advanced treatment strategies for CF.

cystic fibrosis  /  cystic fibrosis transmembrane conductance regulator  /  gene mutation  /  CFTR modulator  /  mechanism of action of drugs  /  antibiotic
Lin LIN, Yan WANG, Chuan-bin WU, Chun-e ZHU. Research progress of cystic fibrosis and its therapies[J]. Acta Pharmaceutica Sinica, 2022 , 57 (7) : 2024 -2031 . DOI: 10.16438/j.0513-4870.2021-1750
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Article Info
doi: 10.16438/j.0513-4870.2021-1750
  • Receive Date：2021-12-07
  • Online Date：2025-12-23
  • Published：2022-07-12
Article Data
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History
  • Received：2021-12-07
  • Revised：2022-01-29
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Affiliations
    1. School of Chinese Materia Medica, Guangdong Pharmaceutical University, Guangzhou 510006, China
    2. School of Pharmaceutical Sciences, Jinan University, Guangzhou 510032, China
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表12种不同金属材料的力学参数
科
Family		 属数
Number of
genus		 种数
Number of
species		 占总种数比例
Percentage of
total species (%)		 属
Genus		 种数
Number of
species		 占总种数比例
Percentage of total
species (%)
鹅膏菌科Amanitaceae 2 11 5.26 鹅膏菌属 Amanita 10 4.78
小菇科 Mycenaceae 2 12 5.74 丝盖伞属 Inocybe 5 2.39
多孔菌科 Polyporaceae 8 14 6.70 蜡蘑属 Laccaria 5 2.39
红菇科 Russulaceae 3 23 11.00 小皮伞属 Marasmius 6 2.87
小菇属 Mycena 11 5.26
光柄菇属 Pluteus 5 2.39
红菇属 Russula 17 8.13
栓菌属 Trametes 5 2.39
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