Aberrant Accumulation of Fibulin-3 in the Endoplasmic Reticulum Leads to Activation of the Unfolded Protein Response and VEGF Expression

The inherited early-onset macular degenerative disease known as malattia leventinese (ML) and Doyne honeycomb retinal dystrophy (DHRD) have been linked to a missense mutation leading to production of a mutant fibulin-3 protein (R345W). R345W is poorly secreted and accumulates in the RPE of ML/DHRD r...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Investigative ophthalmology & visual science 2005-11, Vol.46 (11), p.3973-3979
Hauptverfasser:	Roybal, C. Nathaniel, Marmorstein, Lihua Y, Vander Jagt, David L, Abcouwer, Steve F
Format:	Artikel
Sprache:	eng
Schlagworte:	Adenoviridae - genetics Biological and medical sciences Blotting, Northern Blotting, Western Cell Line DNA-Binding Proteins - genetics DNA-Binding Proteins - metabolism Endoplasmic Reticulum - metabolism Extracellular Matrix Proteins - physiology Eye and associated structures. Visual pathways and centers. Vision Fluorescent Antibody Technique, Indirect Fundamental and applied biological sciences. Psychology Genetic Vectors Heat-Shock Proteins - genetics Heat-Shock Proteins - metabolism Humans Molecular Chaperones - genetics Molecular Chaperones - metabolism Nuclear Proteins - genetics Nuclear Proteins - metabolism Pigment Epithelium of Eye - metabolism Plasmids Promoter Regions, Genetic Regulatory Factor X Transcription Factors Reverse Transcriptase Polymerase Chain Reaction RNA, Messenger - metabolism Transcription Factors Up-Regulation Vascular Endothelial Growth Factor A - biosynthesis Vascular Endothelial Growth Factor A - genetics Vertebrates: nervous system and sense organs X-Box Binding Protein 1
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Schreiben Sie den ersten Kommentar!