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Background  Loss of proprotein convertase subtilisin/kexin type 5 ( Pcsk5 ) results in multiple developmental anomalies including cardiac malformations, caudal regression, pre-sacral mass, renal agenesis, anteroposterior patterning defects, and tracheo-oesophageal and anorectal malformations, and is a model for VACTERL/caudal regression/Currarino syndromes (VACTERL association - Vertebral anomalies, Anal atresia, Cardiac defects, Tracheoesophageal fistula and/or Esophageal atresia, Renal & Radial anomalies and Limb defects).    Results  Using magnetic resonance imaging (MRI), we examined heart development in mouse embryos with zygotic and cardiac specific deletion of  Pcsk5 . We show that conditional deletion of  Pcsk5  in all epiblastic lineages recapitulates all developmental malformations except for tracheo-esophageal malformations. Using a conditional deletion strategy, we find that there is an essential and specific requirement for  Pcsk5  in the cranio-cardiac mesoderm for cardiogenesis, but not for conotruncal septation or any other aspect of embryonic development. Surprisingly, deletion of  Pcsk5  in cardiogenic or pharyngeal mesodermal progenitors that form later from the cranio-cardiac mesoderm does not affect heart development. Neither is  Pcsk5  essential in the neural crest, which drives conotruncal septation.    Conclusions  Our results suggest that  Pcsk5  may have an essential and early role in the cranio-cardiac mesoderm for heart development. Alternatively, it is possible that  Pcsk5  may still play a critical role in  Nkx2.5 -expressing cardiac progenitors, with persistence of mRNA or protein accounting for the lack of effect of deletion on heart development.    Electronic supplementary material  The online version of this article (doi:10.1186/s12861-017-0148-y) contains supplementary material, which is available to authorized users.

Pcsk5 is required in the early cranio-cardiac mesoderm for heart development