Page 1 |
Save page Remove page | Previous | 1 of 158 | Next |
|
small (250x250 max)
medium (500x500 max)
large ( > 500x500)
Full Resolution
All (PDF)
|
This page
All
Subset |
ESSENTIAL ROLE OF THE CARBOXYL-TERMINUS FOR PROPER RHODOPSIN TRAFFICKING AND “ENLIGHTENMENT” TO THE PATHWAY(S) CAUSING RETINAL DEGENERATION IN A MOUSE MODEL EXPRESSING A TRUNCATED RHODOPSIN MUTANT
by
Francis Avila Concepcion
____________________________________________________________________
A Dissertation Presented to the
FACULTY OF THE GRADUATE SCHOOL
UNIVERSITY OF SOUTHERN CALIFORNIA
In Partial Fulfillment of the
Requirements for the Degree
DOCTOR OF PHILOSOPHY
(CELL AND NEUROBIOLOGY)
December 2007
Copyright 2007 Francis Avila Concepcion
Object Description
| Title | Essential role of the carboxyl-terminus for proper rhodopsin trafficking and "enlightenment" to the pathway(s) causing retinal degeneration in a mouse model expressing a truncated rhodopsin mutant |
| Author | Concepcion, Francis Avila |
| Author email | faconcepcion@gmail.com |
| Degree | Doctor of Philosophy |
| Document type | Dissertation |
| Degree program | Cell & Neurobiology |
| School | Keck School of Medicine |
| Date defended/completed | 2007-08-13 |
| Date submitted | 2007 |
| Restricted until | Unrestricted |
| Date published | 2007-09-05 |
| Advisor (committee chair) | Chen, Jeannie |
| Advisor (committee member) |
Garner, Judy Chow, Robert Okamoto, Curtis |
| Abstract | Retinitis pigmentosa (RP) is a heterogeneous group of inherited eye diseases that is typified by initial night blindness and loss of peripheral vision and eventually culminates into total blindness. This retinal disorder afflicts around 1 in 4000 people worldwide. Mutations in the carboxyl-terminus of rhodopsin have been linked to autosomal dominant RP (ADRP). In this study, we have investigated two truncated rhodopsin mutants, S334ter and Q344ter. Both mutants have demonstrated that the presence of the QVAPA domain is necessary for proper rhodopsin localization and ROS formation. However, the retention of the phosphorylation sites in the Q344ter (and not in the S334ter) has allowed us to demonstrate the light-activation capability of mislocalized rhodopsin. More pertinent to ADRP patients, the retention of these phosphorylation sites in Q344ter also contributes to light-accelerated retinal degeneration through a mechanism first described in Drosophila, the accumulation of the rhodopsin/arrestin complexes in abnormal subcellular compartments. |
| Keyword | ADRP; rhodopsin transport; rod photoreceptor; Q344ter; S334ter; retinal degeneration |
| Language | English |
| Part of collection | University of Southern California dissertations and theses |
| Publisher (of the original version) | University of Southern California |
| Place of publication (of the original version) | Los Angeles, California |
| Publisher (of the digital version) | University of Southern California. Libraries |
| Type | texts |
| Legacy record ID | usctheses-m806 |
| Rights | Concepcion, Francis Avila |
| Repository name | Libraries, University of Southern California |
| Repository address | Los Angeles, California |
| Repository email | http://www.usc.edu/isd/libraries/services/ask_a_librarian/email/ |
| Filename | etd-Concepcion-20070905 |
| Archival file | uscthesesreloadpub_Volume23/etd-Concepcion-20070905.pdf |
Description
| Title | Page 1 |
| Full text | ESSENTIAL ROLE OF THE CARBOXYL-TERMINUS FOR PROPER RHODOPSIN TRAFFICKING AND “ENLIGHTENMENT” TO THE PATHWAY(S) CAUSING RETINAL DEGENERATION IN A MOUSE MODEL EXPRESSING A TRUNCATED RHODOPSIN MUTANT by Francis Avila Concepcion ____________________________________________________________________ A Dissertation Presented to the FACULTY OF THE GRADUATE SCHOOL UNIVERSITY OF SOUTHERN CALIFORNIA In Partial Fulfillment of the Requirements for the Degree DOCTOR OF PHILOSOPHY (CELL AND NEUROBIOLOGY) December 2007 Copyright 2007 Francis Avila Concepcion |
Comments
Post a Comment for Page 1
