Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes

Mitochondria are surrounded by two membranes; the outer mitochondrial membrane and the inner mitochondrial membrane. They are unique organelles since they have their own DNA, the mitochondrial DNA (mtDNA), which is replicated continuously. Mitochondrial membranes have direct interaction with mtDNA a...

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Main Authors: Mohammed Almannai, Azza Salah, Ayman W. El-Hattab
Format: Article
Language:English
Published: MDPI AG 2022-06-01
Series:Membranes
Subjects:
Online Access:https://www.mdpi.com/2077-0375/12/6/625
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author Mohammed Almannai
Azza Salah
Ayman W. El-Hattab
author_facet Mohammed Almannai
Azza Salah
Ayman W. El-Hattab
author_sort Mohammed Almannai
collection DOAJ
description Mitochondria are surrounded by two membranes; the outer mitochondrial membrane and the inner mitochondrial membrane. They are unique organelles since they have their own DNA, the mitochondrial DNA (mtDNA), which is replicated continuously. Mitochondrial membranes have direct interaction with mtDNA and are therefore involved in organization of the mitochondrial genome. They also play essential roles in mitochondrial dynamics and the supply of nucleotides for mtDNA synthesis. In this review, we will discuss how the mitochondrial membranes interact with mtDNA and how this interaction is essential for mtDNA maintenance. We will review different mtDNA maintenance disorders that result from defects in this crucial interaction. Finally, we will review therapeutic approaches relevant to defects in mitochondrial membranes.
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spelling doaj.art-b0c002bc66704a0c8e0e1dd53a4ed0242023-11-23T17:55:01ZengMDPI AGMembranes2077-03752022-06-0112662510.3390/membranes12060625Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical SyndromesMohammed Almannai0Azza Salah1Ayman W. El-Hattab2Genetics and Precision Medicine Department, King Abdullah Specialized Children Hospital, Riyadh P.O. Box 22490, Saudi ArabiaDepartment of Pediatrics, University Hospital Sharjah, Sharjah P.O. Box 72772, United Arab EmiratesDepartment of Clinical Sciences, College of Medicine, University of Sharjah, Sharjah P.O. Box 27272, United Arab EmiratesMitochondria are surrounded by two membranes; the outer mitochondrial membrane and the inner mitochondrial membrane. They are unique organelles since they have their own DNA, the mitochondrial DNA (mtDNA), which is replicated continuously. Mitochondrial membranes have direct interaction with mtDNA and are therefore involved in organization of the mitochondrial genome. They also play essential roles in mitochondrial dynamics and the supply of nucleotides for mtDNA synthesis. In this review, we will discuss how the mitochondrial membranes interact with mtDNA and how this interaction is essential for mtDNA maintenance. We will review different mtDNA maintenance disorders that result from defects in this crucial interaction. Finally, we will review therapeutic approaches relevant to defects in mitochondrial membranes.https://www.mdpi.com/2077-0375/12/6/625mitochondriaIMMOMMfissionfusionmtDNA
spellingShingle Mohammed Almannai
Azza Salah
Ayman W. El-Hattab
Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes
Membranes
mitochondria
IMM
OMM
fission
fusion
mtDNA
title Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes
title_full Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes
title_fullStr Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes
title_full_unstemmed Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes
title_short Mitochondrial Membranes and Mitochondrial Genome: Interactions and Clinical Syndromes
title_sort mitochondrial membranes and mitochondrial genome interactions and clinical syndromes
topic mitochondria
IMM
OMM
fission
fusion
mtDNA
url https://www.mdpi.com/2077-0375/12/6/625
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