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Abnormal respiratory motile cilium physiology

MedGen UID:
868585
Concept ID:
C4022984
Pathologic Function
HPO: HP:0012261

Definition

Any functional anomaly of the respiratory motile cilia. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal respiratory motile cilium physiology

Professional guidelines

PubMed

Zhou MH, Lin Y, Zhang ZG
Biochim Biophys Acta Mol Cell Res 2020 Jul;1867(7):118641. Epub 2019 Dec 30 doi: 10.1016/j.bbamcr.2019.118641. PMID: 31893523
Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, Dell S, Eber E, Escudier E, Hirst RA, Hogg C, Jorissen M, Latzin P, Legendre M, Leigh MW, Midulla F, Nielsen KG, Omran H, Papon JF, Pohunek P, Redfern B, Rigau D, Rindlisbacher B, Santamaria F, Shoemark A, Snijders D, Tonia T, Titieni A, Walker WT, Werner C, Bush A, Kuehni CE
Eur Respir J 2017 Jan;49(1) Epub 2017 Jan 4 doi: 10.1183/13993003.01090-2016. PMID: 27836958Free PMC Article
Lobo J, Zariwala MA, Noone PG
Semin Respir Crit Care Med 2015 Apr;36(2):169-79. Epub 2015 Mar 31 doi: 10.1055/s-0035-1546748. PMID: 25826585Free PMC Article

Recent clinical studies

Etiology

Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y
Hum Reprod Update 2023 May 2;29(3):347-367. doi: 10.1093/humupd/dmad003. PMID: 36721921Free PMC Article
Wallmeier J, Nielsen KG, Kuehni CE, Lucas JS, Leigh MW, Zariwala MA, Omran H
Nat Rev Dis Primers 2020 Sep 17;6(1):77. doi: 10.1038/s41572-020-0209-6. PMID: 32943623
Lucas JS, Davis SD, Omran H, Shoemark A
Lancet Respir Med 2020 Feb;8(2):202-216. Epub 2019 Oct 14 doi: 10.1016/S2213-2600(19)30374-1. PMID: 31624012
Harrison MJ, Shapiro AJ, Kennedy MP
Paediatr Respir Rev 2016 Mar;18:25-32. Epub 2015 Sep 26 doi: 10.1016/j.prrv.2015.09.003. PMID: 26545972
Afzelius BA
J Pathol 2004 Nov;204(4):470-7. doi: 10.1002/path.1652. PMID: 15495266Free PMC Article

Diagnosis

Despotes KA, Zariwala MA, Davis SD, Ferkol TW
Cells 2024 Jun 4;13(11) doi: 10.3390/cells13110974. PMID: 38891105Free PMC Article
Wee WB, Kinghorn B, Davis SD, Ferkol TW, Shapiro AJ
Pediatrics 2024 Jun 1;153(6) doi: 10.1542/peds.2023-063064. PMID: 38695103Free PMC Article
Shoemark A, Griffin H, Wheway G, Hogg C, Lucas JS; Genomics England Research Consortium, Camps C, Taylor J, Carroll M, Loebinger MR, Chalmers JD, Morris-Rosendahl D, Mitchison HM, De Soyza A; Genomics England Research Consortium:, Brown D, Ambrose JC, Arumugam P, Bevers R, Bleda M, Boardman-Pretty F, Boustred CR, Brittain H, Caulfield MJ, Chan GC, Fowler T, Giess A, Hamblin A, Henderson S, Hubbard TJP, Jackson R, Jones LJ, Kasperaviciute D, Kayikci M, Kousathanas A, Lahnstein L, Leigh SEA, Leong IUS, Lopez FJ, Maleady-Crowe F, McEntagart M, Minneci F, Moutsianas L, Mueller M, Murugaesu N, Need AC, O'Donovan P, Odhams CA, Patch C, Perez-Gil D, Pereira MB, Pullinger J, Rahim T, Rendon A, Rogers T, Savage K, Sawant K, Scott RH, Siddiq A, Sieghart A, Smith SC, Sosinsky A, Stuckey A, Tanguy M, Taylor Tavares AL, Thomas ERA, Thompson SR, Tucci A, Welland MJ, Williams E, Witkowska K, Wood SM
Eur Respir J 2022 Nov;60(5) Epub 2022 Nov 17 doi: 10.1183/13993003.00176-2022. PMID: 35728977
Wallmeier J, Nielsen KG, Kuehni CE, Lucas JS, Leigh MW, Zariwala MA, Omran H
Nat Rev Dis Primers 2020 Sep 17;6(1):77. doi: 10.1038/s41572-020-0209-6. PMID: 32943623
Lucas JS, Davis SD, Omran H, Shoemark A
Lancet Respir Med 2020 Feb;8(2):202-216. Epub 2019 Oct 14 doi: 10.1016/S2213-2600(19)30374-1. PMID: 31624012

Therapy

Paff T, Omran H, Nielsen KG, Haarman EG
Int J Mol Sci 2021 Sep 11;22(18) doi: 10.3390/ijms22189834. PMID: 34575997Free PMC Article
Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, Dell S, Eber E, Escudier E, Hirst RA, Hogg C, Jorissen M, Latzin P, Legendre M, Leigh MW, Midulla F, Nielsen KG, Omran H, Papon JF, Pohunek P, Redfern B, Rigau D, Rindlisbacher B, Santamaria F, Shoemark A, Snijders D, Tonia T, Titieni A, Walker WT, Werner C, Bush A, Kuehni CE
Eur Respir J 2017 Jan;49(1) Epub 2017 Jan 4 doi: 10.1183/13993003.01090-2016. PMID: 27836958Free PMC Article
Knowles MR, Zariwala M, Leigh M
Clin Chest Med 2016 Sep;37(3):449-61. Epub 2016 Jun 30 doi: 10.1016/j.ccm.2016.04.008. PMID: 27514592Free PMC Article
Lobo J, Zariwala MA, Noone PG
Semin Respir Crit Care Med 2015 Apr;36(2):169-79. Epub 2015 Mar 31 doi: 10.1055/s-0035-1546748. PMID: 25826585Free PMC Article
Afzelius BA
J Pathol 2004 Nov;204(4):470-7. doi: 10.1002/path.1652. PMID: 15495266Free PMC Article

Prognosis

Walton T, Gui M, Velkova S, Fassad MR, Hirst RA, Haarman E, O'Callaghan C, Bottier M, Burgoyne T, Mitchison HM, Brown A
Nature 2023 Jun;618(7965):625-633. Epub 2023 May 31 doi: 10.1038/s41586-023-06140-2. PMID: 37258679Free PMC Article
Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y
Hum Reprod Update 2023 May 2;29(3):347-367. doi: 10.1093/humupd/dmad003. PMID: 36721921Free PMC Article
Lobo J, Zariwala MA, Noone PG
Semin Respir Crit Care Med 2015 Apr;36(2):169-79. Epub 2015 Mar 31 doi: 10.1055/s-0035-1546748. PMID: 25826585Free PMC Article
Gras D, Chanez P, Vachier I, Petit A, Bourdin A
Pharmacol Ther 2013 Dec;140(3):290-305. Epub 2013 Jul 21 doi: 10.1016/j.pharmthera.2013.07.008. PMID: 23880290
Vervoort R, Wright AF
Hum Mutat 2002 May;19(5):486-500. doi: 10.1002/humu.10057. PMID: 11968081

Clinical prediction guides

Walton T, Gui M, Velkova S, Fassad MR, Hirst RA, Haarman E, O'Callaghan C, Bottier M, Burgoyne T, Mitchison HM, Brown A
Nature 2023 Jun;618(7965):625-633. Epub 2023 May 31 doi: 10.1038/s41586-023-06140-2. PMID: 37258679Free PMC Article
Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y
Hum Reprod Update 2023 May 2;29(3):347-367. doi: 10.1093/humupd/dmad003. PMID: 36721921Free PMC Article
Shoemark A, Griffin H, Wheway G, Hogg C, Lucas JS; Genomics England Research Consortium, Camps C, Taylor J, Carroll M, Loebinger MR, Chalmers JD, Morris-Rosendahl D, Mitchison HM, De Soyza A; Genomics England Research Consortium:, Brown D, Ambrose JC, Arumugam P, Bevers R, Bleda M, Boardman-Pretty F, Boustred CR, Brittain H, Caulfield MJ, Chan GC, Fowler T, Giess A, Hamblin A, Henderson S, Hubbard TJP, Jackson R, Jones LJ, Kasperaviciute D, Kayikci M, Kousathanas A, Lahnstein L, Leigh SEA, Leong IUS, Lopez FJ, Maleady-Crowe F, McEntagart M, Minneci F, Moutsianas L, Mueller M, Murugaesu N, Need AC, O'Donovan P, Odhams CA, Patch C, Perez-Gil D, Pereira MB, Pullinger J, Rahim T, Rendon A, Rogers T, Savage K, Sawant K, Scott RH, Siddiq A, Sieghart A, Smith SC, Sosinsky A, Stuckey A, Tanguy M, Taylor Tavares AL, Thomas ERA, Thompson SR, Tucci A, Welland MJ, Williams E, Witkowska K, Wood SM
Eur Respir J 2022 Nov;60(5) Epub 2022 Nov 17 doi: 10.1183/13993003.00176-2022. PMID: 35728977
Davis SD, Rosenfeld M, Lee HS, Ferkol TW, Sagel SD, Dell SD, Milla C, Pittman JE, Shapiro AJ, Sullivan KM, Nykamp KR, Krischer JP, Zariwala MA, Knowles MR, Leigh MW
Am J Respir Crit Care Med 2019 Jan 15;199(2):190-198. doi: 10.1164/rccm.201803-0548OC. PMID: 30067075Free PMC Article
Vervoort R, Wright AF
Hum Mutat 2002 May;19(5):486-500. doi: 10.1002/humu.10057. PMID: 11968081

Recent systematic reviews

Peng B, Gao YH, Xie JQ, He XW, Wang CC, Xu JF, Zhang GJ
Orphanet J Rare Dis 2022 Jul 19;17(1):283. doi: 10.1186/s13023-022-02427-1. PMID: 35854386Free PMC Article
Inaba A, Furuhata M, Morimoto K, Rahman M, Takahashi O, Hijikata M, Knowles MR, Keicho N
BMC Pulm Med 2019 Jul 25;19(1):135. doi: 10.1186/s12890-019-0897-4. PMID: 31345208Free PMC Article

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