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Schnyder corneal dystrophy-associated UBIAD1 is defective in MK-4 synthesis and resists autophagy-mediated degradation

The autosomal dominant disorder Schnyder corneal dystrophy (SCD) is caused by mutations in UbiA prenyltransferase domain-containing protein-1 (UBIAD1), which uses geranylgeranyl pyrophosphate (GGpp) to synthesize the vitamin K(2) subtype menaquinone-4 (MK-4). SCD is characterized by opacification of...

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Detalhes bibliográficos
Publicado no:J Lipid Res
Main Authors: Jun, Dong-Jae, Schumacher, Marc M., Hwang, Seonghwan, Kinch, Lisa N., Grishin, Nick V., DeBose-Boyd, Russell A.
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society for Biochemistry and Molecular Biology 2020
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7193952/
https://ncbi.nlm.nih.gov/pubmed/32188638
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.RA119000551
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