The incubation of adrenal ferredoxin with various detergents in the presence of oxygen or ferricyanide leads to bleaching of the protein. The bleached preparation has the properties of apoferredoxin and it can be reconstituted with high yield by conventional methods.
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http://dx.doi.org/10.1042/bj1530725 | DOI Listing |
J Clin Endocrinol Metab
November 2024
Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern 3010, Switzerland.
JCI Insight
June 2024
Division of Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics, Inselspital, Bern University Hospital, and.
Genetic defects affecting steroid biosynthesis cause cortisol deficiency and differences of sex development; among these defects are recessive mutations in the steroidogenic enzymes CYP11A1 and CYP11B, whose function is supported by reducing equivalents donated by ferredoxin reductase (FDXR) and ferredoxin. So far, mutations in the mitochondrial flavoprotein FDXR have been associated with a progressive neuropathic mitochondriopathy named FDXR-related mitochondriopathy (FRM), but cortisol insufficiency has not been documented. However, patients with FRM often experience worsening or demise following stress associated with infections.
View Article and Find Full Text PDFSheng Wu Gong Cheng Xue Bao
November 2023
College of Biotechnology, Tianjin University of Science & Technology, Tianjin 300457, China.
17α hydroxylase is a key enzyme for the conversion of progesterone to prepare various progestational drug intermediates. To improve the specific hydroxylation capability of this enzyme in steroid biocatalysis, the CYP260A1 derived from cellulose-mucilaginous bacteria Soce56 and the Fpr and bovine adrenal-derived Adx derived from str. K-12 were used to construct a new electron transfer system for the conversion of progesterone.
View Article and Find Full Text PDFJ Biol Chem
July 2022
Department of Pharmacology and Pharmaceutical Sciences, School of Pharmacy, University of Southern California, Los Angeles, California, USA. Electronic address:
Neurosteroids, modulators of neuronal and glial cell functions, are synthesized in the nervous system from cholesterol. In peripheral steroidogenic tissues, cholesterol is converted to the major steroid precursor pregnenolone by the CYP11A1 enzyme. Although pregnenolone is one of the most abundant neurosteroids in the brain, expression of CYP11A1 is difficult to detect.
View Article and Find Full Text PDFAnn Pediatr Endocrinol Metab
September 2021
Department of Pediatrics, Center for Reproductive Sciences and Institute for Human Genetics, University of California, San Francisco, CA, USA.
Most steroidogenesis disorders are caused by mutations in genes encoding the steroidogenic enzymes, but work in the past 20 years has identified related disorders caused by mutations in the genes encoding the cofactors that transport electrons from NADPH to P450 enzymes. Most P450s are microsomal and require electron donation by P450 oxidoreductase (POR); by contrast, mitochondrial P450s require electron donation via ferredoxin reductase (FdxR) and ferredoxin (Fdx). POR deficiency is the most common and best-described of these new forms of congenital adrenal hyperplasia.
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