Structure of a Membrane-Embedded Prenyltransferase Homologous to UBIAD1

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Structure of a Membrane-Embedded Prenyltransferase Homologous to UBIAD1

Membrane-embedded prenyltransferases from the UbiA family catalyze the Mg2+-dependent transfer of a hydrophobic polyprenyl chain onto a variety of acceptor molecules and are involved in the synthesis of molecules that mediate electron transport, including Vitamin K and Coenzyme Q. In humans, missense mutations to the protein UbiA prenyltransferase domain-containing 1 (UBIAD1) are responsible fo...

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UBIAD1 Mutation Alters a Mitochondrial Prenyltransferase to Cause Schnyder Corneal Dystrophy

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The prenyltransferase UBIAD1 is the target of geranylgeraniol in degradation of HMG CoA reductase

Schnyder corneal dystrophy (SCD) is an autosomal dominant disorder in humans characterized by abnormal accumulation of cholesterol in the cornea. SCD-associated mutations have been identified in the gene encoding UBIAD1, a prenyltransferase that synthesizes vitamin K2. Here, we show that sterols stimulate binding of UBIAD1 to the cholesterol biosynthetic enzyme HMG CoA reductase, which is subje...

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ژورنال

عنوان ژورنال: PLoS Biology

سال: 2014

ISSN: 1545-7885

DOI: 10.1371/journal.pbio.1001911