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Volume 4, Issue 3 (Aug 2019)
JNFS 2019, 4(3): 206-217 |
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Ghasemzadeh N, Karimi-Nazari E, Yaghoubi F, Zarei S, Azadmanesh F, Sargazi S. Molybdenum Cofactor Biology and Disorders Related to Its Deficiency; A Review Study . JNFS 2019; 4 (3) :206-217
URL: http://jnfs.ssu.ac.ir/article-1-278-en.html
URL: http://jnfs.ssu.ac.ir/article-1-278-en.html
Navid Ghasemzadeh 
, Elham Karimi-Nazari , Fatemeh Yaghoubi , Sadegh Zarei , Fatemeh Azadmanesh , Saman Sargazi *
, Elham Karimi-Nazari , Fatemeh Yaghoubi , Sadegh Zarei , Fatemeh Azadmanesh , Saman Sargazi *
Cellular and Molecular Research Center, Zahedan University of Medical Sciences, Zahedan, Iran.
Abstract: (2539 Views)
Background: Molybden, as a vital and essential micronutrient is directly involved in the metabolism of other elements including carbon, sulfur, and nitrogen. Molybdenum alone is not biologically active unless it binds to specific cofactors. Except for the bacterial nitrogenase, which contains molybdenum-Iron complex, molybdenum cofactor (Moco) is considered as the bioactive component placed in active site regions of molybdenum-containing enzymes. This review aimed to discuss the biological mechanisms involved in molybdenum metabolism highlighting Molybdenum cofactor deficiencies. Methods: Articles indexed in Pubmed, Google Scholar, and Scopus databases were used to extract the required information. Results: Moco, as the cofactor of sulfite oxidase, xanthine dehydrogenase, aldehyde oxidase, and nitrite reductase plays a substantial role in maintaining normal body homeostasis and reactive oxygen species (ROS) production. Lack of Moco is found to be associated with many inborn genetic disorders, such as mental retardation, brain immaturity, nervous shocks, and neurodegenerative diseases. Conclusion: Moco insufficiency compromises normal human body metabolism since it is reported to regulate the metabolic pathways of other elements. Although in recent years, substitution- and gene-therapies have been introduced to restore the metabolic pathways of patients with MoCD type A and B, the definitive treatment for this type of inborn disease has still remained ill-defined. More investigations are needed to completely understand the underlying pathophysiology of molybdenum-related diseases.
Type of article: review article |
Subject:
public specific
Received: 2018/10/1 | Published: 2019/08/1 | ePublished: 2019/08/1
Received: 2018/10/1 | Published: 2019/08/1 | ePublished: 2019/08/1
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