KEGG   DISEASE: 嚢胞性肺疾患
エントリ  
H02906                                                             
名称    
嚢胞性肺疾患
概要    
Polycystic lung disease (PCLUD) is characterized by multiple intrapulmonary cysts. A lung cyst is an air-filled lucent structure surrounded by a thin wall. It has been reported that inherited CCR2 deficiency underlies progressive polycystic lung disease. CCR2 is the main receptor for chemokine C-C motif ligand 2 (CCL-2), which is expressed on human monocytes, dendritic cells, and basophils. It is suggested that CCR2 is of particular importance for the recruitment and maturation of macrophages in the lungs.
カテゴリ  
呼吸器系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  主に1つの体系に影響する構造的発達異常
   呼吸器系の構造的発達異常
    LA75  肺の構造的発達異常
     H02906  嚢胞性肺疾患
病因遺伝子 
CCR2 [HSA:729230] [KO:K04177]
リンク   
ICD-11: LA75.4
OMIM: 219600
文献    
  著者
Neehus AL, Carey B, Landekic M, Panikulam P, Deutsch G, Ogishi M, Arango-Franco CA, Philippot Q, Modaresi M, Mohammadzadeh I, Corcini Berndt M, Rinchai D, Le Voyer T, Rosain J, Momenilandi M, Martin-Fernandez M, Khan T, Bohlen J, Han JE, Deslys A, Bernard M, Gajardo-Carrasco T, Soudee C, Le Floc'h C, Migaud M, Seeleuthner Y, Jang MS, Nikolouli E, Seyedpour S, Begueret H, Emile JF, Le Guen P, Tavazzi G, Colombo CNJ, Marzani FC, Angelini M, Trespidi F, Ghirardello S, Alipour N, Molitor A, Carapito R, Mazloomrezaei M, Rokni-Zadeh H, Changi-Ashtiani M, Brouzes C, Vargas P, Borghesi A, Lachmann N, Bahram S, Crestani B, Fayon M, Galode F, Pahari S, Schlesinger LS, Marr N, Bogunovic D, Boisson-Dupuis S, Beziat V, Abel L, Borie R, Young LR, Deterding R, Shahrooei M, Rezaei N, Parvaneh N, Craven D, Gros P, Malo D, Sepulveda FE, Nogee LM, Aladjidi N, Trapnell BC, Casanova JL, Bustamante J
  タイトル
Human inherited CCR2 deficiency underlies progressive polycystic lung disease.
  雑誌
Cell 187:390-408.e23 (2024)
DOI:10.1016/j.cell.2023.11.036
文献    
  著者
Koo HK, Yoo CG
  タイトル
Multiple cystic lung disease.
  雑誌
Tuberc Respir Dis (Seoul) 74:97-103 (2013)
DOI:10.4046/trd.2013.74.3.97
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