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Title

Coenzyme Q biosynthesis is regulated by RNA-protein interaction

AuthorsCascajo Almenara, M. V. CSIC ORCID; Siendones, Emilio CSIC ORCID; Willers, Imke M.; Cuezva, José M. CSIC ORCID; Navas, Plácido CSIC ORCID
Issue Date2012
Citation22nd IUBMB and 37th FEBS (2012)
AbstractCoenzyme Q (CoQ) deficiency is a rare disorder with a variable phenotypic presentation that includes pure myopathy, myopathy with encephalopathy, cerebellar atrophy with ataxia, and infantile multisystem disease including encephalopathy and nephrophaty, and nephritic syndrome. Primary CoQ deficiency arises from mutations in COQ genes, while secondary forms of CoQ deficiency are caused by mutations in genes not involved in CoQ biosynthesis. In most patients, the exact site and nature of the defects on biosynthesis have not yet been identified. Because CoQ biosynthesis is complex and not fully defined, identification of the molecular genetic defects has been challenging. At least ten genes (COQ1-COQ10) forming a multi-peptide complex are required for CoQ biosynthesis. One of them, COQ7, is a central regulator of the pathway. We have previously demonstrated that NF-kB regulatesCOQ7 gene transcription under oxidative stress. Our current studies have uncovered the interaction of the RNAbinding protein (RBP) HuR and other as-yet unidentified RBPs with the 3'UTR region of the COQ7 mRNA. We propose a model of post-transcriptional regulation of COQ7 expression, whereby RBPs binding to the COQ7 3'UTR can rapidly and effectively alter COQ7 expression levels to adapt to changing cellular needs for cellular CoQ activity.
DescriptionResumen del póster presentado al 22nd IUBMB & 37th FEBS Congress, celebrado en Sevilla (España) del 4 al 9 de septiembre de 2012.-- et al.
URIhttp://hdl.handle.net/10261/130143
Appears in Collections:(CBM) Comunicaciones congresos
(CABD) Comunicaciones congresos
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