Редактирование: ARIH2

E3 ubiquitin-protein ligase ARIH2 (EC 2.3.2.31) (ARI-2) (Protein ariadne-2 homolog) (RING-type E3 ubiquitin transferase ARIH2) (Triad1 protein) [ARI2] [TRIAD1] [HT005]

==Publications==

{{medline-entry
|title=A novel feed-forward loop between [[ARIH2]] E3-ligase and [[PABPN1]] regulates aging-associated muscle degeneration.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/24486325
|abstract=Alanine expansion mutations in poly(A)-binding protein nuclear 1 ([[PABPN1]]) cause muscle weakness in the late-onset disorder oculopharyngeal muscular dystrophy. In affected muscles, expanded [[PABPN1]] forms nuclear aggregates, depleting levels of soluble [[PABPN1]] and inducing a genome-wide shift from distal to proximal polyadenylation site usage. [[PABPN1]] protein accumulation is regulated by the ubiquitin proteasome system, which is highly dysregulated in oculopharyngeal muscular dystrophy. We show that [[ARIH2]] E3-ligase regulates [[PABPN1]] protein accumulation and aggregation. Levels of [[ARIH2]] mRNA are regulated by [[PABPN1]] via proximal polyadenylation site usage. We demonstrate that masking the proximal polyadenylation site in [[ARIH2]] 3' untranslated region by antisense oligonucleotides elevates the expression of [[ARIH2]] and [[PABPN1]] and restores myogenic defects that are induced by [[ARIH2]] or [[PABPN1]] down-regulation in cell culture. In vivo [[ARIH2]] mRNA levels significantly decrease from midlife in vastus lateralis muscles and highly correlate with muscle degeneration. We suggest that the expression of both genes is maintained by a feed-forward loop between mRNA stability regulated by [[PABPN1]] and protein turnover regulated by [[ARIH2]]. 
|mesh-terms=* Aging
* Animals
* Blotting, Western
* Cell Line
* Gene Expression Regulation
* Humans
* Immunohistochemistry
* Immunoprecipitation
* Muscle, Skeletal
* Muscular Dystrophy, Oculopharyngeal
* Oligonucleotide Array Sequence Analysis
* Poly(A)-Binding Protein I
* Reverse Transcriptase Polymerase Chain Reaction
* Transfection
* Ubiquitin-Protein Ligases

|full-text-url=https://sci-hub.do/10.1016/j.ajpath.2013.12.011
}}