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Dev Biol 2019 Dec 01;4561:1-7. doi: 10.1016/j.ydbio.2019.07.019.
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Alkylglycerol monooxygenase, a heterotaxy candidate gene, regulates left-right patterning via Wnt signaling.

Duncan AR , González DP , Del Viso F , Robson A , Khokha MK , Griffin JN .

Congenital heart disease (CHD) is a major cause of morbidity in the pediatric population yet its genetic and molecular causes remain poorly defined. Previously, we identified AGMO as a candidate heterotaxy disease gene, a disorder of left-right (LR) patterning that can have a profound effect on cardiac function. AGMO is the only known alkylglycerol monooxygenase, an orphan tetrahydrobiopterin dependent enzyme that cleaves the ether linkage in alkylglycerols. However, whether AGMO plays a role in LR patterning was unexplored. Here we reveal that Agmo is required for correct development of the embryonic LR axis in Xenopus embryos recapitulating the patient's heterotaxy phenotype. Mechanistically, we demonstrate that Agmo is a regulator of canonical Wnt signaling, required during gastrulation for normal formation of the left - right organizer. Mutational analysis demonstrates that this function is dependent on Agmo's alkylglycerol monooxygenase activity. Together, our findings identify Agmo as a regulator of canonical Wnt signaling, demonstrate a role for Agmo in embryonic axis formation, and provide insight into the poorly understood developmental requirements for ether lipid cleavage.

PubMed ID: 31398317
PMC ID: PMC7784330
Article link: Dev Biol
Grant support: [+]

Species referenced: Xenopus
Genes referenced: agmo ctnnb1 dand5 gdf3 nodal1
GO keywords: Wnt signaling pathway

Disease Ontology terms: visceral heterotaxy [+]

Article Images: [+] show captions
References [+] :
Alrayes, The alkylglycerol monooxygenase (AGMO) gene previously involved in autism also causes a novel syndromic form of primary microcephaly in a consanguineous Saudi family. 2016, Pubmed