Universität zu Köln

Schwarz, Guenter ORCID: 0000-0002-2118-9338 (2016). Molybdenum cofactor and human disease. Curr. Opin. Chem. Biol., 31. S. 179 - 188. OXFORD: ELSEVIER SCI LTD. ISSN 1879-0402

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Abstract

Four molybdenum-dependent enzymes are known in humans, each harboring a pterin-based molybdenum cofactor (Moco) in the active site. They catalyze redox reactions using water as oxygen acceptor or donator. Moco is synthesized by a conserved biosynthetic pathway. Moco deficiency results in a severe inborn error of metabolism causing often early childhood death. Disease-causing symptoms mainly go back to the lack of sulfite oxidase (SO) activity, an enzyme in cysteine catabolism. Besides their name-giving functions, Mo-enzymes have been recognized to catalyze novel reactions, including the reduction of nitrite to nitric oxide. In this review we cover the biosynthesis of Moco, key features of Moco-enzymes and focus on their deficiency. Underlying disease mechanisms as well as treatment options will be discussed.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Schwarz, Guenter UNSPECIFIED orcid.org/0000-0002-2118-9338 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-280377
DOI:	10.1016/j.cbpa.2016.03.016
Journal or Publication Title:	Curr. Opin. Chem. Biol.
Volume:	31
Page Range:	S. 179 - 188
Date:	2016
Publisher:	ELSEVIER SCI LTD
Place of Publication:	OXFORD
ISSN:	1879-0402
Language:	English
Faculty:	Faculty of Mathematics and Natural Sciences
Divisions:	Faculty of Mathematics and Natural Sciences > Department of Chemistry > Institute of Biochemistry
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CYCLIC PYRANOPTERIN MONOPHOSPHATE; SULFITE OXIDASE DEFICIENCY; CYSTATHIONINE GAMMA-LYASE; NITRIC-OXIDE; HEREDITARY XANTHINURIA; HUNTINGTONS-DISEASE; ALDEHYDE OXIDASE; S-SULFOCYSTEINE; ENZYMES; GEPHYRIN Multiple languages Biochemistry & Molecular Biology; Biophysics Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/28037