Stains & CD markers
Cytochrome c oxidase (COX)
Copyright: 2016-2024, PathologyOutlines.com, Inc.
PubMed Search: Cytochrome oxidase [title] stains
Cytochrome c oxidase (COX)
Author: Sheren Younes, M.D., Ph.D.
Editorial Board Member: Lauren N. Stuart, M.D., M.B.A.
Last author update: 1 October 2016
Last staff update: 17 June 2021
Copyright: 2016-2024, PathologyOutlines.com, Inc.
PubMed Search: Cytochrome oxidase [title] stains
Cite this page: Younes S. Cytochrome c oxidase (COX). PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/stainscox.html. Accessed November 26th, 2024.
Definition / general
- Also referred to as Complex IV
- Unique terminal oxidase of the mitochondrial respiratory chain in mammals
- Located at the inner mitochondrial membrane
- Catalyzes the transfer of electrons from ferrocytochrome c to molecular oxygen, for proton transfer across the inner mitochondrial membrane and ATP synthesis
- Complex made up of 13 subunits
- Three large, catalytic subunits are encoded with mitochondrial DNA
- Contain all the heme and metal prosthetic groups needed for catalysis
- The remaining 10 subunits are encoded by nuclear DNA and transported to mitochondria
- COX biosynthesis requires many assembly factors that are not a part of the final complex
- Various isoforms of COX exist according to tissue and developmental stage
- Exists in an active form as a dimer
- Several prosthetic groups are required for its catalytic function: 2 heme groups, 2 copper centers, zinc and magnesium
Diagrams / tables
Images hosted on other servers:
Synthesis and assembly of COX subunits
Maturation and insertion of COX into the respiratory chain
Clinical features
-
Diseases associated with COX malfunction:
- Varies according to the affected gene; examples include mitochondrial disease, neurodegenerative disease, aging and tumors
- Mutations or deficiencies in either COX genes or assembly genes may result in disease:
- MTCO1 gene deficiency - encephalomyopathy (Mitochondrion 2014;17:101), prostatic carcinoma (Proc Natl Acad Sci U S A 2005;102:719)
- MTCO2 gene deficiency - cardiomyopathy (Clin Exp Pharmacol Physiol 2009;36:933)
- COX19 deficiency - non-small cell lung carcinoma (Int J Clin Exp Med 2015;8:8835)
- SOC2 - breast carcinoma (Oncotarget 2015;6:43363)
