Impaired hepatic drug and steroid metabolism in congenital adrenal hyperplasia due to P450 oxidoreductase deficiency [PDF]
Objective: Patients with congenital adrenal hyperplasia due to P450 oxidoreductase (POR) deficiency(ORD) present with disordered sex development and glucocorticoid deficiency.
Arlt +25 more
core +2 more sources
The emergence of resistance to azole drugs, presented significant problems for medicine that could be overcome by advances in antifungal design. We convinced that the crystal structures of fungal and host lanosterol-14α-demethylases in complex with a ...
Hamid Irannejad +3 more
doaj +1 more source
A conserved amino acid residue critical for product and substrate specificity in plant triterpene synthases [PDF]
Triterpenes are structurally complex plant natural products with numerous medicinal applications. They are synthesized through an origami-like process that involves cyclization of the linear 30 carbon precursor 2,3-oxidosqualene into different triterpene
Al-Qurainy +12 more
core +1 more source
Mutations in the _SC4MOL_ gene encoding a novel methyl sterol oxidase cause autosomal recessive psoriasisiform dermatitis, microcephaly and developmental delay [PDF]
Disorders of cholesterol biosynthesis have clinical manifestations involving skeleton, eyes, neurologic development, and skin. We describe a patient with congenital cataracts, developmental delay, microcephaly, and low serum cholesterol who developed ...
Abbe Vallejo +8 more
core +1 more source
The Investigational Drug VT-1129 Is a Highly Potent Inhibitor of Cryptococcus Species CYP51 but Only Weakly Inhibits the Human Enzyme [PDF]
Cryptococcosis is a life-threatening disease often associated with HIV infection. Three Cryptococcus species CYP51 enzymes were purified and catalyzed the 14α-demethylation of lanosterol, eburicol, and obtusifoliol.
Garvey, Edward P. +8 more
core +1 more source
Does litomosoides sigmodontis synthesize dimethylethanolamine from choline? [PDF]
Juvenile female Litomosoides sigmodontis secrete a protein (Juv-p120) highly modified with dimethylethanolamine (DMAE). In an attempt to establish the source of this decoration worms were pulsed with [3H]-choline and [3H]-ethanolamine and the radio ...
Allen, J E +4 more
core +2 more sources
Jaborandi (Pilocarpus microphyllus) essential oil is obtained by removal and characterization using GC‐MS, revealing γ‐cadinene and trans‐caryophyllene as major constituents. The oil exhibits biological activity, with antifungal action against Candida spp.
Juniel Cruz Silva +15 more
wiley +1 more source
The late steps of cholesterol biosynthesis are oxygen demanding, requiring eleven oxygen molecules per synthesized cholesterol molecule. A key enzymatic reaction, which occurs at the top of the Bloch and Kandutsch-Russell pathways, is the demethylation ...
Jun Zhu +2 more
doaj +1 more source
Metabolic and pathologic profiles of human LSS deficiency recapitulated in mice.
Skin lesions, cataracts, and congenital anomalies have been frequently associated with inherited deficiencies in enzymes that synthesize cholesterol. Lanosterol synthase (LSS) converts (S)-2,3-epoxysqualene to lanosterol in the cholesterol biosynthesis ...
Yoichi Wada +18 more
doaj +1 more source
Genetic engineering and molecular characterization of yeast strain expressing hybrid human-yeast squalene synthase as a tool for anti-cholesterol drug assessment [PDF]
AIMS: The main objective of the study is molecular and biological characterization of the human-yeast hybrid squalene synthase (SQS), as a promising target for treatment of hypercholesterolaemia.
Burzynska, Beata +12 more
core +1 more source

