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Hand2 null mouse embryos suffer lethality around E11.0 due to absence of a right ventricle and lack of aortic arch arteries (Srivastava et al., 1997).
Mef2c null mice suffer lethality between E9.5 and E10.5 due to inability to undergo cardiac looping, absence of the right ventricle, atrial and ventricular hypoplasia, reduced trabeculation and shortened AVC with absent ECCs.
Tbx5 null mice suffer lethality by E10.5 due to looping abnormalities, failure to form two atria and hypoplasia of the single atrium and left ventricle (Bruneau et al., 2001).
Tbx2 null mice suffer lethality by E14.5 demonstrating abnormal AVC morphology, severely retarded ECC growth, OFT septation defects and ectopic expression of chamber-specific genes Nppa and Smpx (Harrelson et al., 2004).
Par3 null mouse embryos suffer lethality from E10.5 to E12.5 because a loss of cell polarity results in a failure to form the vesicles that bud from the PEO, causing subsequent absence of epicardium in mouse hearts.
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Those who survive have substantial disability, most often never achieving independent ambulation and usually requiring continuous ventilator support, and suffer premature lethality.
Homozygous Gata4 deficient mice suffer embryonic lethality between E7.0 and E9.5 and fail to form the linear heart tube due to inappropriate lateral to ventral embryonic folding and extraembyonic defects (Kuo et al., 1997; Molkentin et al., 1997).
This explains the female-specific inheritance of FDH., Mutant males suffer embryonic lethality, whereas mutant females survive and exhibit variable clinical manifestations as a result of random X-chromosome inactivation.
However, null homozygotes suffered embryonic lethality at E10.5-11.5 due to compromised cardiovascular development that was attributed to elevated Notch1 expression.
Cdx2 is a TE-specific transcription factor that is critical for specifying TE [33], [34]; Cdx2-null embryos undergo apoptosis within the TE and suffer from preimplantation lethality [34].
C57BL/6- Lrrk1tm1.1Mjff/J KO mice suffer from neonatal lethality and reasons for phenotype differences resulting from the two different KO strategies are unclear.
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