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Enzymes are proteins made of amino acids. The functional group of each constituent amino acid catalyzes a wide variety of chemical reactions via ionic interactions or acid-base reactions. However, amino acids cannot catalyze oxidation-reduction and group transfer reactions and need to be aided by non-protein components called cofactors. Cofactors are also referred to as the chemical teeth of an enzyme.
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Enzymes require additional components for proper function. There are two such classes of molecules: cofactors and coenzymes. Cofactors are metallic ions and coenzymes are non-protein organic molecules. Both of these types of helper molecule can be tightly bound to the enzyme or bound only when the substrate binds.
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Molybdenum cofactor and human disease.

Guenter Schwarz1

  • 1Institute of Biochemistry, Department of Chemistry and Center for Molecular Medicine Cologne and Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases, University of Cologne, Zuelpicher Str. 47, 50674 Koeln, Germany.

Current Opinion in Chemical Biology
|April 8, 2016
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Summary
This summary is machine-generated.

Molybdenum cofactor (Moco) is vital for human enzymes catalyzing redox reactions. Moco deficiency causes severe metabolic disorders, primarily impacting sulfite oxidase (SO) activity, leading to early death.

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Area of Science:

  • Biochemistry
  • Enzymology
  • Metabolic Disorders

Background:

  • Molybdenum-dependent enzymes utilize a pterin-based molybdenum cofactor (Moco).
  • These enzymes catalyze essential redox reactions, including those involving water and nitrite.
  • Moco biosynthesis follows a conserved pathway crucial for enzyme function.

Purpose of the Study:

  • To review Moco biosynthesis and the characteristics of Moco-dependent enzymes.
  • To focus on Moco deficiency, its underlying disease mechanisms, and potential treatments.
  • To highlight novel functions of Moco-enzymes, such as nitrite reduction.

Main Methods:

  • Literature review of Moco biosynthesis.
  • Analysis of Moco-enzyme functions and deficiencies.
  • Discussion of disease mechanisms and therapeutic strategies.

Main Results:

  • Moco is essential for four known human enzymes involved in redox reactions.
  • Moco deficiency leads to severe metabolic diseases, often fatal in childhood, mainly due to absent sulfite oxidase (SO) activity.
  • Moco-enzymes also perform novel functions, like the reduction of nitrite to nitric oxide.

Conclusions:

  • Moco-dependent enzymes are critical for human health, with Moco deficiency representing a severe metabolic disorder.
  • Understanding Moco biosynthesis and enzyme function is key to addressing these diseases.
  • Further research into novel enzyme functions and treatment options is warranted.