A mouse model of conduction system patterning abnormalities in heterotaxy syndrome

Pediatric Research

Volumn 68, Issue 4, 2010, Pages 275-280

  Czosek, Richard J ^a   Haaning, Allison ^a   Ware, Stephanie M ^a  

^a UNIVERSITY OF CINCINNATI COLLEGE OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

COMPLEMENTARY DNA; DIGOXIGENIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONGENITAL HEART DISEASE; CONTROLLED STUDY; EMBRYO; HEART DEVELOPMENT; HEART MUSCLE CONDUCTION DISTURBANCE; HEART MUSCLE CONDUCTION SYSTEM; HETEROTAXY SYNDROME; IN SITU HYBRIDIZATION; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; PRIORITY JOURNAL; RNA PROBE;

ANIMALS; BODY PATTERNING; DEXTROCARDIA; DISEASE MODELS, ANIMAL; GENE EXPRESSION REGULATION, DEVELOPMENTAL; GENETIC DISEASES, X-LINKED; GESTATIONAL AGE; HEART CONDUCTION SYSTEM; HEART VENTRICLES; HOMEODOMAIN PROTEINS; HUMANS; MICE; MICE, KNOCKOUT; SEVERITY OF ILLNESS INDEX; SINOATRIAL NODE; SITUS INVERSUS; TRANSCRIPTION FACTORS;

EID: 77957263717     PISSN: 00313998     EISSN: 15300447     Source Type: Journal    
DOI: 10.1203/PDR.0b013e3181ee0028     Document Type: Article

References (36)

1. Ramsdell AF 2005 Left-right asymmetry and congenital cardiac defects: getting to the heart of the matter in vertebrate left-right axis determination. Dev Biol 288:1-20
2. Sutherland MJ, Ware SM 2009 Disorders of left-right asymmetry: heterotaxy and situs inversus. Am J Med Genet C Semin Med Genet 151C:307-317
3. Belmont JW, Mohapatra B, Towbin JA, Ware SM 2004 Molecular genetics of heterotaxy syndromes. Curr Opin Cardiol 19:216-220
4. Jacobs JP, O'Brien SM, Chai PJ, Morell VO, Lindberg HL, Quintessenza JA 2008 Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007. Ann Thorac Surg 85:1691-1696; discussion 1697
5. Wilkinson JL, Smith A, Lincoln C, Anderson RH 1978 Conducting tissues in congenitally corrected transposition with situs inversus. Br Heart J 40:41-48
6. Cheung YF, Cheng VY, Yung TC, Chau AK 2002 Cardiac rhythm and symptomatic arrhythmia in right atrial isomerism. Am Heart J 144:159-164
7. Epstein MR, Saul JP, Weindling SN, Triedman JK, Walsh EP 2001 Atrioventricular reciprocating tachycardia involving twin atrioventricular nodes in patients with complex congenital heart disease. J Cardiovasc Electrophysiol 12:671-679
8. Wu MH, Wang JK, Lin JL, Lai LP, Lue HC, Hsieh FJ 2001 Cardiac rhythm disturbances in patients with left atrial isomerism. Pacing Clin Electrophysiol 24:1631-1638
9. Gourdie RG, Harris BS, Bond J, Justus C, Hewett KW, O'Brien TX, Thompson RP, Sedmera D 2003 Development of the cardiac pacemaking and conduction system. Birth Defects Res C Embryo Today 69:46-57
10. Moorman AF, De Jong F, Denyn MM, Lamers WH 1998 Development of the cardiac conduction system. Circ Res 82:629-644
11. Rentschler S, Vaidya DM, Tamaddon H, Degenhardt K, Sassoon D, Morley GE, Jalife J, Fishman GI 2001 Visualization and functional characterization of the developing murine cardiac conduction system. Development 128:1785-1792 (Pubitemid 32514858)
12. Kondo RP, Anderson RH, Kupershmidt S, Roden DM, Evans SM 2003 Development of the cardiac conduction system as delineated by minK-lacZ. J Cardiovasc Electrophysiol 14:383-391
13. Jongbloed MR, Schalij MJ, Poelmann RE, Blom NA, Fekkes ML, Wang Z, Fishman GI, Gittenberger-De Groot AC 2004 Embryonic conduction tissue: a spatial correlation with adult arrhythmogenic areas. J Cardiovasc Electrophysiol 15:349-355
14. Jongbloed MR, Wijffels MC, Schalij MJ, Blom NA, Poelmann RE, Van Der Laarse A, Mentink MM, Wang Z, Fishman GI, Gittenberger-de Groot AC 2005 Development of the right ventricular inflow tract and moderator band: a possible morphological and functional explanation for Mahaim tachycardia. Circ Res 96:776-783
15. Myers DC, Fishman GI 2004 Toward an understanding of the genetics of murine cardiac pacemaking and conduction system development. Anat Rec A Discov Mol Cell Evol Biol 280:1018-1021
16. Purandare SM, Ware SM, Kwan KM, Gebbia M, Bassi MT, Deng JM, Vogel H, Behringer RR, Belmont JW, Casey B 2002 A complex syndrome of left-right axis, central nervous system and axial skeleton defects in Zic3 mutant mice. Development 129:2293-2302
17. Ware SM, Harutyunyan KG, Belmont JW 2006 Heart defects in X-linked hetero-taxy: evidence for a genetic interaction of Zic3 with the nodal signaling pathway. Dev Dyn 235:1631-1637
18. Ware SM, Harutyunyan KG, Belmont JW 2006 Zic3 is critical for early embryonic patterning during gastrulation. Dev Dyn 235:776-785
19. Ware SM, Peng J, Zhu L, Fernbach S, Colicos S, Casey B, Towbin J, Belmont JW 2004 Identification and functional analysis of ZIC3 mutations in heterotaxy and related congenital heart defects. Am J Hum Genet 74:93-105
20. MacGregor GR, Nolan GP, Fiering S, Roederer M, Herzenberg LA 1991 Use of E. Coli lacZ (/3-Galactosidase) as a reporter gene. In: Murray EJ (ed) Gene Transfer and Expression Protocols. The Humana Press Inc., Clifton, pp 217-235
21. Santoro B, Chen S, Luthi A, Pavlidis P, Shumyatsky GP, Tibbs GR, Siegelbaum SA 2000 Molecular and functional heterogeneity of hyperpolarization-activated pacemaker channels in the mouse CNS. J Neurosci 20:5264-5275
22. Searcy RD, Vincent EB, Liberatore CM, Yutzey KE 1998 A GATA-dependent nkx-2.5 regulatory element activates early cardiac gene expression in transgenic mice. Development 125:4461-4470
23. Shelton EL, Yutzey KE 2007 Tbx20 regulation of endocardial cushion cell proliferation and extracellular matrix gene expression. Dev Biol 302:376-388
24. Hoogaars WM, Engel A, Brons JF, Verkerk AO, De Lange FJ, Wong LY, Bakker ML, Clout DE, Wakker V, Barnett P, Ravesloot JH, Moorman AF, Verheijck EE, Christoffels VM 2007 Tbx3 controls the sinoatrial node gene program and imposes pacemaker function on the atria. Genes Dev 21:1098-1112
25. Hoogaars WM, Tessari A, Moorman AF, De Boer PA, Hagoort J, Soufan AT, Campione M, Christoffels VM 2004 The transcriptional repressor Tbx3 delineates the developing central conduction system of the heart. Cardiovasc Res 62:489-499
26. Mommersteeg MT, Hoogaars WM, Prall OW, De Gier-de Vries C, Wiese C, Clout DE, Papaioannou VE, Brown NA, Harvey RP, Moorman AF, Christoffels VM 2007 Molecular pathway for the localized formation of the sinoatrial node. Circ Res 100:354-362
27. Dickinson DF, Wilkinson JL, Anderson KR, Smith A, Ho SY, Anderson RH 1979 The cardiac conduction system in situs ambiguus. Circulation 59:879-885
28. Ho SY, Seo JW, Brown NA, Cook AC, Fagg NL, Anderson RH 1995 Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages. Br Heart J 74:437-442
29. Cohen MS, Anderson RH, Cohen MI, Atz AM, Fogel M, Gruber PJ, Lopez L, Rome JJ, Weinberg PM 2007 Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young 17:29-43
30. Blaschke RJ, Hahurij ND, Kuijper S, Just S, Wisse LJ, Deissler K, Maxelon T, Anastassiadis K, Spitzer J, Hardt SE, Scholer H, Feitsma H, Rottbauer W, Blum M, Meijlink F, Rappold G, Gittenberger-de Groot AC 2007 Targeted mutation reveals essential functions of the homeodomain transcription factor Shox2 in sinoatrial and pacemaking development. Circulation 115:1830-1838
31. Berul CI, Aronovitz MJ, Wang PJ, Mendelsohn ME 1996 In vivo cardiac electro-physiology studies in the mouse. Circulation 94:2641-2648
32. Bae EJ, Noh CI, Choi JY, Yun YS, Kim WH, Lee JR, Kim YJ 2005 Twin AV node and induced supraventricular tachycardia in Fontan palliation patients. Pacing Clin Electrophysiol 28:126-134
33. Gurjarpadhye A, Hewett KW, Justus C, Wen X, Stadt H, Kirby ML, Sedmera D, Gourdie RG 2007 Cardiac neural crest ablation inhibits compaction and electrical function of conduction system bundles. Am J Physiol Heart Circ Physiol 292:H1291-H1300
34. Bharati S, Lev M 1975 The course of the conduction system in single ventricle with inverted (L-) loop and inverted (L-) transposition. Circulation 51:723-730
35. Meno C, Shimono A, Saijoh Y, Yashiro K, Mochida K, Ohishi S, Noji S, Kondoh H, Hamada H 1998 lefty-1 is required for left-right determination as a regulator of lefty-2 and nodal. Cell 94:287-297
36. Tessari A, Pietrobon M, Notte A, Cifelli G, Gage PJ, Schneider MD, Lembo G, Campione M 2008 Myocardial Pitx2 differentially regulates the left atrial identity and ventricular asymmetric remodeling programs. Circ Res 102:813-822