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The FMRpolyGlycine protein mediates aggregate formation and toxicity independent of the CGG mRNA hairpin in a cellular model for FXTAS
(Journal article; Tidsskriftartikkel; Peer reviewed, 2019-03-28)
Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by a CGG-repeat expansion in the 5′ UTR of the FMR1 gene on the X-chromosome. Both elevated levels of the expanded FMR1 mRNA and aberrant expression of a polyglycine protein (FMRpolyG) from the CGG-repeat region are hypothesized to trigger the pathogenesis of FXTAS. While increased expression of FMRpolyG leads ...
Pax6 Represses Androgen Receptor-Mediated Transactivation by Inhibiting Recruitment of the Coactivator SPBP
(Journal article; Tidsskriftartikkel; Peer reviewed, 2011)
The androgen receptor (AR) has a central role in development and maintenance of the male reproductive system and in the etiology of prostate cancer. The transcription factor Pax6 has recently been reported to act as a repressor of AR and to be hypermethylated in prostate cancer cells. SPBP is a transcriptional regulator that previously has been shown to enhance the activity of Pax6. In this study ...
SPBP is a sulforaphane induced transcriptional coactivator of NRF2 regulating expression of the autophagy receptor p62/SQSTM1
(Journal article; Tidsskriftartikkel; Peer reviewed, 2014)
Transforming growth factor-beta-inducible early response gene 1 is a novel substrate for atypical protein kinase Cs
(Journal article; Tidsskriftartikkel; Peer reviewed, 2011)
The protein kinase C (PKC) family of serine/threonine kinases consists of ten different isoforms grouped into three subfamilies, denoted classical, novel and atypical PKCs (aPKCs). The aPKCs, PKC?/? and PKC? serve important roles during development and in processes subverted in cancer such as cell and tissue polarity, cell proliferation, differentiation and apoptosis. In an effort to identify novel ...
A Phylogenetic Study of SPBP and RAI1: Evolutionary Conservation of Chromatin Binding Modules
(Journal article; Tidsskriftartikkel; Peer reviewed, 2013)
Our genome is assembled into and array of highly dynamic nucleosome structures allowing spatial and temporal
access to DNA. The nucleosomes are subject to a wide array of post-translational modifications, altering the DNAhistone
interaction and serving as docking sites for proteins exhibiting effector or “reader” modules. The nuclear
proteins SPBP and RAI1 are composed of several putative “reader” ...
P62/sequestosome-1, autophagy-related gene 8, and autophagy in Drosophila are regulated by nuclear factor erythroid 2-related factor 2(NRF2), independent of transcription factor TFEB
(Journal article; Tidsskriftartikkel; Peer reviewed, 2015-04-30)
The selective autophagy receptor p62/sequestosome 1 (SQSTM1) interacts directly with LC3 and is involved in oxidative stress signaling in two ways in mammals. First, p62 is transcriptionally induced upon oxidative stress by the NF-E2-related factor 2 (NRF2) by direct binding to an antioxidant response element in the p62 promoter. Second, p62 accumulation, occurring when autophagy is impaired, leads ...
CALCOCO1 acts with VAMP ‐associated proteins to mediate ER ‐phagy
(Journal article; Tidsskriftartikkel; Peer reviewed, 2020-06-11)
The endoplasmic reticulum (ER) plays important roles in protein synthesis and folding, and calcium storage. The volume of the ER and expression of its resident proteins are increased in response to nutrient stress. ER‐phagy, a selective form of autophagy, is involved in the degradation of the excess components of the ER to restore homeostasis. Six ER‐resident proteins have been identified as ER‐phagy ...
NIMA-related kinase 9–mediated phosphorylation of the microtubule-associated LC3B protein at Thr-50 suppresses selective autophagy of p62/sequestosome 1
(Journal article; Tidsskriftartikkel; Peer reviewed, 2019-12-19)
Human ATG8 family proteins (ATG8s) are active in all steps of the macroautophagy pathway, and their lipidation is essential for autophagosome formation. Lipidated ATG8s anchored to the outer surface of the phagophore serve as scaffolds for binding of other core autophagy proteins and various effector proteins involved in trafficking or fusion events, whereas those at the inner surface are needed for ...
TRIM27 is an autophagy substrate facilitating mitochondria clustering and mitophagy via phosphorylated TBK1
(Journal article; Tidsskriftartikkel; Peer reviewed, 2022-09-16)
Tripartite motif-containing protein 27 (TRIM27/also called RFP) is a multifunctional ubiquitin E3 ligase involved in numerous cellular functions, such as proliferation, apoptosis, regulation of the NF-kB pathway, endosomal recycling and the innate immune response. TRIM27 interacts directly with TANK-binding kinase 1 (TBK1) and regulates its stability. TBK1 in complex with autophagy receptors is ...