KEGG   DISEASE: Polycystic lung disease
Entry
H02906                      Disease                                
Name
Polycystic lung disease
Description
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.
Category
Respiratory system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 20 Developmental anomalies
  Structural developmental anomalies primarily affecting one body system
   Structural developmental anomalies of the respiratory system
    LA75  Structural developmental anomalies of lungs
     H02906  Polycystic lung disease
Gene
CCR2 [HSA:729230] [KO:K04177]
Other DBs
ICD-11: LA75.4
OMIM: 219600
Reference
  Authors
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
  Title
Human inherited CCR2 deficiency underlies progressive polycystic lung disease.
  Journal
Cell 187:390-408.e23 (2024)
DOI:10.1016/j.cell.2023.11.036
Reference
  Authors
Koo HK, Yoo CG
  Title
Multiple cystic lung disease.
  Journal
Tuberc Respir Dis (Seoul) 74:97-103 (2013)
DOI:10.4046/trd.2013.74.3.97
LinkDB

» Japanese version

DBGET integrated database retrieval system