Редактирование: PQBP1

Polyglutamine-binding protein 1 (PQBP-1) (38 kDa nuclear protein containing a WW domain) (Npw38) (Polyglutamine tract-binding protein 1) [NPW38] [JM26]

==Publications==

{{medline-entry
|title=A restricted level of [[PQBP1]] is needed for the best longevity of Drosophila.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/22901698
|abstract=A number of neurological diseases are caused by mutations of RNA metabolism-related genes. A complicating issue is that whether under- or overfunction of such genes is responsible for the phenotype. Polyglutamine tract binding protein-1, a causative gene for X-linked mental retardation, is also involved in RNA metabolism, and both mutation and duplication of the gene were reported in human patients. In this study, we first report a novel phenotype of d[[PQBP1]] (drosophila homolog of Polyglutamine tract binding protein-1)-mutant flies, lifespan shortening. We next address the gene dose-phenotype relationship in lifespan shortening and in learning disability, a previously described phenotype. The 2 phenotypes are rescued by d[[PQBP1]] but in different dose-phenotype relationships. Either insufficient or excessive expression of d[[PQBP1]] does not recover lifespan, while excessive expression recovers learning ability. We finally address the mechanism of lifespan shortening. Tissue-specific expression of d[[PQBP1]]-RNA interference construct reveals both neural and nonneural d[[PQBP1]] contribute to the lifespan, while the latter has a dominant effect. Gene expression profiling suggested retinophilin/MORN repeat containing 4, a gene promoting axonal degeneration, to contribute to lifespan shortening by neural d[[PQBP1]]. Systems biology analysis of the gene expression profiles revealed indirect influence of d[[PQBP1]] on insulin-like growth factor 1, insulin receptor, and peroxisome proliferator-activated receptorα/γ signaling pathways in nonneural tissues. Collectively, given that d[[PQBP1]] affects multiple pathways in different dose-dependent and tissue-specific manners, d[[PQBP1]] at a restricted expression level is needed for the best longevity.
|mesh-terms=* Age Factors
* Animals
* Animals, Genetically Modified
* Caenorhabditis elegans
* Carrier Proteins
* DNA-Binding Proteins
* Drosophila
* Enzyme Inhibitors
* Gene Regulatory Networks
* Humans
* Hydroxamic Acids
* Learning Disabilities
* Longevity
* Mutation
* Neuroglia
* Neurons
* Nuclear Proteins
* Phenotype

|full-text-url=https://sci-hub.do/10.1016/j.neurobiolaging.2012.07.015
}}