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Citations to this article

Molybdenum cofactor biosynthesis in humans. Identification of two complementation groups of cofactor-deficient patients and preliminary characterization of a diffusible molybdopterin precursor.
J L Johnson, … , R Mandell, V E Shih
J L Johnson, … , R Mandell, V E Shih
Published March 1, 1989
Citation Information: J Clin Invest. 1989;83(3):897-903. https://doi.org/10.1172/JCI113974.
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Research Article

Molybdenum cofactor biosynthesis in humans. Identification of two complementation groups of cofactor-deficient patients and preliminary characterization of a diffusible molybdopterin precursor.

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Abstract

Molybdenum cofactor deficiency is a devastating disease with affected patients displaying the symptoms of a combined deficiency of sulfite oxidase and xanthine dehydrogenase. Because of the extreme lability of the isolated, functional molybdenum cofactor, direct cofactor replacement therapy is not feasible, and a search for stable biosynthetic intermediates was undertaken. From studies of cocultured fibroblasts from affected individuals, two complementation groups were identified. Coculture of group A and group B cells, without heterokaryon formation, led to the appearance of active sulfite oxidase. Use of conditioned media indicated that a relatively stable, diffusible precursor produced by group B cells could be used to repair sulfite oxidase in group A recipient cells. Although the extremely low levels of precursor produced by group B cells preclude its direct characterization, studies with a heterologous, in vitro reconstitution system suggest that the precursor that accumulates in group B cells is the same as a molybdopterin precursor identified in the Neurospora crassa molybdopterin mutant nit-1, and that a converting enzyme is present in group A cells which catalyzes an activation reaction analogous to that of a converting enzyme identified in the Escherichia coli molybdopterin mutant ChlA1.

Authors

J L Johnson, M M Wuebbens, R Mandell, V E Shih

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Total citations by year

Year: 2023 2022 2019 2018 2017 2014 2013 2012 2011 2010 2007 2006 2005 2003 2002 2001 2000 1999 1998 1995 1994 1993 1992 1991 1990 1989 Total
Citations: 1 1 2 1 1 1 2 1 2 5 1 1 1 3 3 1 1 4 3 4 3 3 1 3 2 1 52
Citation information
This citation data is accumulated from CrossRef, which receives citation information from participating publishers, including this journal. Not all publishers participate in CrossRef, so this information is not comprehensive. Additionally, data may not reflect the most current citations to this article, and the data may differ from citation information available from other sources (for example, Google Scholar, Web of Science, and Scopus).

Citations to this article in year 2011 (2)

Title and authors Publication Year
Advances in Clinical Chemistry
M Mahler
Advances in Clinical Chemistry Volume 54 2011
Structure and stability of the molybdenum cofactor intermediate cyclic pyranopterin monophosphate
JA Santamaria-Araujo, V Wray, G Schwarz
JBIC Journal of Biological Inorganic Chemistry 2011

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