TABLE 2.

Selected differentially expressed liver genes from human BA, man1a2 morphant zebrafish and Man1a2^–/– mouse (see Supplementary Tables 9–12 for complete list).

Pathway	HUMAN BA Liver	Man1a2–/– mouse liver	man1a2 morphant zebrafish
HEDGEHOG	GPC1	Gpc1, Ptch1	gli1*
EGF	FGF23, FGF2, FGF7	Egfr, Fgf23, Asap1	egfra*
TGF	TGFB2	Smad9, Smad1, Smad3, Smad4, Tgfbr2	smad9*, smad1, smad7*
Selected CILIARY genes (SYS-CILIA GOLD)	MAN1A2*, DYNC2H1, DNAH11, IFT80, IFT81	Man1a2, Dync2h1, Dnah2, Ift74, Invs	hap1
Human PRIMARY CILIARY DYSKINESIS genes with laterality	ACVR2B, CENPF, DNAH11, GATA4, HYDIN, OFD1, RPGR	Bcl9l, Ccno, Cenpf, Cluap1, Dnaaf2, Drc1, Hydin, Invs, Rpgr,
CONGENITAL HEART DISEASE heterotaxy genes with extracardiac effects	EHMT1, MID1, RUNX1, GATA4	Ankrd11, Camta1, Crebbp, Crk, Ehmt1, Flna, Gata6, Gja5, Gtf2Ird1, Hras, Nek2, Notch1, Nrp1, Nsd1, Pde1a, Rock2, Runx1, Sox7, Tgfbr2

These genes participate in developmental hedgehog, EGF, TGF and ciliogenesis pathways, or associate with PCD or congenital heart disease with laterality or extracardiac defects. Bold text identifies dysregulated genes in two or more species. Liver transcriptome was characterized with RNA sequencing. (^∗) Genes characterized with RTqPCR.