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Molecular Mechanisms of Organelle Biogenesis and Related Metabolic Diseases

Dhaunsi G.S.

Author affiliations

Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait

Corresponding Author

Dr. Gursev S. Dhaunsi

Department of Pediatrics, Faculty of Medicine, Kuwait University

PO Box 24923

13110 Safat (Kuwait)

Tel. +965 531 9486, Fax +965 533 8940, E-Mail dhaunsig@hsc.edu.kw

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Med Princ Pract 2005;14:49–57

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Organelle biogenesis is regulated by transcriptional networks that modulate expression of specific genes encoding organellar proteins. Structural and functional specificity of organelles requires not only the transcription of specific genes and translation of resulting mRNAs, but also the transfer of encoded polypeptides to their site of function through signaling peptides. A defect in targeting of proteins to their subcellular site of function may not necessarily prevent biogenesis of the organelle, but would definitely lead to formation of a defective organelle with respect to that specific function. Several metabolic diseases are associated with dysfunction or defects in organelle biogenesis; among these, peroxisome biogenesis disorders, mitochondrial biogenesis defects and lysosomal storage disorders are an extensively studied group of genetic diseases where biogenesis of the organelle is compromised either due to a defect in assembly of the organelle itself or impaired import of matrix proteins into the organelle. Recent advances in biochemical and molecular aspects of biogenesis of subcellular organelles have not only unraveled the mechanisms for organization of cellular networks, but have also provided new insights into the development of metabolic diseases that are caused by disruption of organelle biogenesis. This article reviews the molecular mechanisms of biogenesis of mitochondria, lysosomes and peroxisomes in relation to the metabolic diseases of genetic or nongenetic origin.

© 2005 S. Karger AG, Basel


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Article / Publication Details

First-Page Preview
Abstract of Review

Received: June 02, 2004
Accepted: December 21, 2004
Published online: July 09, 2008
Issue release date: July 2005

Number of Print Pages: 9
Number of Figures: 0
Number of Tables: 1

ISSN: 1011-7571 (Print)
eISSN: 1423-0151 (Online)

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