Loss of Raf-1 Kinase Inhibitory Protein Delays Early-Onset Severe Retinal Ciliopathy inCep290rd16Mouse
نویسندگان
چکیده
منابع مشابه
Raf kinase inhibitory protein regulates Raf-1 but not B-Raf kinase activation.
Raf kinase inhibitory protein (RKIP; also known as phosphatidylethanolamine-binding protein or PEBP) is a modulator of the Raf/MAPK signaling cascade and a suppressor of metastatic cancer. Here, we show that RKIP inhibits MAPK by regulating Raf-1 activation; specifically, RKIP acts subsequent to Raf-1 membrane recruitment, prevents association of Raf-1 and p21-activated kinase (PAK), and blocks...
متن کاملActivation of Raf-1 signaling by protein kinase C through a mechanism involving Raf kinase inhibitory protein.
Protein kinase C (PKC) regulates activation of the Raf-1 signaling cascade by growth factors, but the mechanism by which this occurs has not been elucidated. Here we report that one mechanism involves dissociation of Raf kinase inhibitory protein (RKIP) from Raf-1. Classic and atypical but not novel PKC isoforms phosphorylate RKIP at serine 153 (Ser-153). RKIP Ser-153 phosphorylation by PKC eit...
متن کاملTubby-like protein 1 homozygous splice-site mutation causes early-onset severe retinal degeneration.
PURPOSE To characterize the disease expression of an autosomal recessive human retinal degeneration associated with a mutation in TULP1 (tubby-like protein 1), a gene with currently unknown function. METHODS Homozygotes and heterozygotes from an extended Dominican kindred with a TULP1 splice-site gene mutation (IVS14+1,G-->A) were studied clinically and with visual function tests. Sequence an...
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We describe a consanguineous Iraqi family with Leber congenital amaurosis (LCA), Joubert syndrome (JBTS), and polycystic kidney disease (PKD). Targeted next-generation sequencing for excluding mutations in known LCA and JBTS genes, homozygosity mapping, and whole-exome sequencing identified a homozygous missense variant, c.317G>C (p.Arg106Pro), in POC1B, a gene essential for ciliogenesis, basal...
متن کاملEnzymatic characteristics of the c-Raf-1 protein kinase.
The c-Raf-1 protein kinase plays a central role in the mitogenic response of cells to growth factors, cytokines, and many oncogenes. Despite the critical importance of this enzyme, very little is known of its biochemical properties or mechanisms of regulation. In these experiments, we used the only candidate physiologic substrate identified as yet for c-Raf-1, mitogen-activated protein kinase k...
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ژورنال
عنوان ژورنال: Investigative Opthalmology & Visual Science
سال: 2014
ISSN: 1552-5783
DOI: 10.1167/iovs.14-14954