Excess Provisional Extracellular Matrix: A Common Factor in Bicuspid Aortic Valve Formation
Abstract
:1. Introduction
2. Development of the Aortic Valve
3. Murine Models of Human BAV-Associated Genes
3.1. Insight of BAV Formation from Mice with Mutations in Notch1 Pathway Genes
3.2. BAV Formation Occurs in Mice with Mutations in Genes That Intersect with Notch Signaling
4. Collation of Murine BAV Data Identifies Common Pathological Intersections in BAV Formation
4.1. Excess P-ECM Is a Common Pathological Finding in Murine BAV Development
4.2. Disruption of CNC Patterning Was Frequently Observed during BAV Formation in Murine Models
5. Proposed Set of Criteria to Evaluate and Compare Murine BAV Models
5.1. Evidence of a BAV Phenotype
5.2. Type of BAV Malformation (LC-RC, LC-NC, RC-NC), Presence or Absence of Raphes (That Indicate Partial Cusp Delineation)
5.3. The Timepoint(s) of AoV Developmental Anomalies
5.4. Cell Lineage Patterning Alterations during OFT Remodeling
5.5. Documentation of Abnormalities in the Ascending Aortic Artery (Aortopathies)
5.6. Characterization of ECM Components Relevant to the Conversion of P-ECM to M-ECM
6. Discussion
Funding
Conflicts of Interest
References
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BAV Gene(s) | BAV % (BAV/Total) | Fusion | >P-ECM | Alt CNC | Alt TGF | Bio Mech | AscAo | Ref |
---|---|---|---|---|---|---|---|---|
Nos3−/−;Notch+/− | 91% (10/11) | NC-RC | T, AB | X | [38,51] | |||
Gata5Cre;Alk2f/f | 80% (12/15) | NC-RC | T, >H | X | none | [52] | ||
Adamts5−/−;Smad2+/− | 77% (10/13) | NC-LC> NC-RC | T, >H, V | X | X | [53,54] | ||
Nfatc1Cre;Ift88f/f | 68% (19/28) | NC-RC | AB, T, >H, V | X | nr | [55] | ||
Isl1Cre;Gata6+/− | 56% M, 27% F* | RC-LC | T, AB | X | X | X | [56] | |
Nkx2.5Cre;Jag1f/f | 47% (7/15) | NC-RC> RC-LC | >H | X | el | [43] | ||
Nfatc1Cre;Exco5f/f | 45% (5/11) | NC-RC>RC-LC | >H | X | X | [57] | ||
Nos3−/− | 42% (5/12) | NC-RC | X | X | none | [38,45] | ||
RockDN, | 42% (13/31) | NC-RC | X | X | [22] | |||
Isl1Cre;Vangl2f/f | 37% (4/7) | No NC | T | X | nr | [41] | ||
Tnnt2Cre;Jag1f/f;Jag2f/f | 36% (4/11) | No NC | >H | nr | [41] | |||
Nfatc1en-Cre;Notch1f/f | 36% (5/14) | NC-RC> RC-LC | T | nr | [43] | |||
Nfatc1Cre;Brg1f/f | 34% (9/36) | NC-LC | T, AB, V | X | nr | [23] | ||
Nfatc1en-Cre;Rbpjf/f | 33% (2/6) | NC-RC | T | nr | [43] | |||
Krox20(Egr2)LacZ/LacZ | 27% (6/27) | NC-RC> RC-LC | T | X | X | nr | [48] | |
Nfatc1enCre;Jag1f/f | 25% (2/8) | NC-RC | >H | nr | [43] | |||
Tie2Cre;Gata5f/f | 25% (7/28) | NC-RC | X | nr | [46] | |||
Hoxa1GFP/GFP | 24% (4/17) | nr | X | el | [58] | |||
Hoxa3-ICre;Fgf8AP/N | 23% (7/33) | RC-NC | X | X | [59] | |||
Robo4tm1Lex/tm1Lex | 20% M, 11% F** | nr | T | X | [34] | |||
Nkx2.5Cre;Mib1f/f | 17% (1/6) | NC-RC | nr | [43] | ||||
Adamts5tm1Dgn/tm1Dgn | 12.5% (3/24) | NC-RC | T, >H, V | X | X | [53,54] | ||
Matr3Gt-Ex13/Gt-Ex13 | 11.5% (3/26) | nr | X | X | [60] | |||
Nkx2-5HDneo | 11%(11/100) | nr | X | nr | [61] | |||
Wnt1Cre;Krox20f/f | 10.5% (2/19) | NC-RC | T | nr | [48] | |||
Tie2Cre;Krox20f/f | 10% (2/20) | nr | nr | [48] | ||||
Npr2+/− | 9.4% (6/64) | NC-RC | T, AB | X | [62] | |||
Notch+/− | 8% (1/12) | NC-RC | X | [38,51] |
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Kern, C.B. Excess Provisional Extracellular Matrix: A Common Factor in Bicuspid Aortic Valve Formation. J. Cardiovasc. Dev. Dis. 2021, 8, 92. https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8080092
Kern CB. Excess Provisional Extracellular Matrix: A Common Factor in Bicuspid Aortic Valve Formation. Journal of Cardiovascular Development and Disease. 2021; 8(8):92. https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8080092
Chicago/Turabian StyleKern, Christine B. 2021. "Excess Provisional Extracellular Matrix: A Common Factor in Bicuspid Aortic Valve Formation" Journal of Cardiovascular Development and Disease 8, no. 8: 92. https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd8080092