Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, is composed of thirteen structural subunits, and requires the addition of several prosthetic groups including, copper and heme moieties, and magnesium and zinc ions. Deficiencies in the assembly of COX lead to several clinical phenotypes including Leigh syndrome and hypertrophic cardiomyopathy. In this study, RNA interference was used in a human cell line to stably knockdown the expression of COX11, a COX assembly factor thought to be involved in Cu B site metallation. These cells showed a reduction in both COX assembly and activity, which were suppressed by overexpression of COX17, but not by SCO1 or SCO2, two proteins involved in CUA metallation. These results support a model of two independent copper delivery pathways to COX, both of which require COX17 as a copper donor.
| Identifer | oai:union.ndltd.org:LACETR/oai:collectionscanada.gc.ca:QMM.81591 |
| Date | January 2004 |
| Creators | Bamji, Michelle |
| Publisher | McGill University |
| Source Sets | Library and Archives Canada ETDs Repository / Centre d'archives des thèses électroniques de Bibliothèque et Archives Canada |
| Language | English |
| Detected Language | English |
| Type | Electronic Thesis or Dissertation |
| Format | application/pdf |
| Coverage | Master of Science (Department of Human Genetics.) |
| Rights | All items in eScholarship@McGill are protected by copyright with all rights reserved unless otherwise indicated. |
| Relation | alephsysno: 002198630, proquestno: AAIMR12394, Theses scanned by UMI/ProQuest. |
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