Results 21 to 30 of about 6,856 (158)

Enzyme activity assay for cholesterol 27-hydroxylase in mitochondria

open access: yesJournal of Lipid Research, 2006
Mitochondrial cholesterol 27-hydroxylase (CYP27A1) plays an important role in the maintenance of intracellular cholesterol homeostasis. Cholesterol delivery to the mitochondrial inner membrane is believed to be a rate-limiting step for the “acidic ...
Xiaobo Li   +3 more
doaj   +1 more source

A novel CYP27A1 frameshift mutation causing cerebrotendinous xanthomatosis in an Indian family

open access: yesAnnals of Movement Disorders, 2022
Cerebrotendinous xanthomatosis is a rare and underreported lipid storage disorder caused by various mutations in the CYP27A1 gene. Here, we report a novel homozygous mutation in the CYP27A1 gene in an Indian family.
Shilpi Shukla   +4 more
doaj   +1 more source

Posttranslational modification by an isolevuglandin diminishes activity of the mitochondrial cytochrome P450 27A1[S]

open access: yesJournal of Lipid Research, 2013
Posttranslational modification by isolevuglandins (isoLGs), arachidonate oxidation products, is an important yet understudied process associated with altered protein properties.
Casey D. Charvet   +4 more
doaj   +1 more source

The Associations of Novel Vitamin D3 Metabolic Gene CYP27A1 Polymorphism, Adiponectin/Leptin Ratio, and Metabolic Syndrome in Middle-Aged Taiwanese Males

open access: yesInternational Journal of Endocrinology, 2015
Metabolic syndrome (MetS) confers increased risks of cardiovascular disease (CVD). Both vitamin D3 and adipocytokines (especially adiponectin and leptin) have a great impact on CVD and MetS.
Kai-Hung Cheng   +10 more
doaj   +1 more source

On the mechanism of accumulation of cholestanol in the brain of mice with a disruption of sterol 27-hydroxylase

open access: yesJournal of Lipid Research, 2010
The rare disease cerebrotendinous xanthomatosis (CTX) is due to a lack of sterol 27-hydroxylase (CYP27A1) and is characterized by cholestanol-containing xanthomas in brain and tendons. Mice with the same defect do not develop xanthomas. The driving force
Ann Båvner   +7 more
doaj   +1 more source

Hematopoietic overexpression of Cyp27a1 reduces hepatic inflammation independently of 27-hydroxycholesterol levels in Ldlr(-/-) mice [PDF]

open access: yes, 2015
Background & Aims: Non-alcoholic steatohepatitis (NASH) is characterized by hepatic lipid accumulation and inflammation. Currently, the underlying mechanisms, leading to hepatic inflammation, are still unknown.
Luetjohann, D.   +14 more
core   +2 more sources

Cerebrotendinous xanthomatosis-a case report with novel compound heterozygous mutation of CYP27A1 gene

open access: yesDermatologica Sinica, 2018
Cerebrotendinous xanthomatosis (CTX), a rare autosomal recessive lipid storage disorder which is caused by mutations in gene CYP27A1 leads to deficiency of enzyme sterol 27-hydroxylase.
Jeng Yuan   +3 more
doaj   +1 more source

TUBB2B facilitates progression of hepatocellular carcinoma by regulating cholesterol metabolism through targeting HNF4A/CYP27A1

open access: yesCell Death and Disease, 2023
Cholesterol metabolism plays a critical role in the progression of hepatocellular carcinoma (HCC), but it is not clear how cholesterol metabolism is regulated.
Xiaobo Wang   +8 more
doaj   +1 more source

Role of CYP27A1 in progesterone metabolism in vitro and in vivo

open access: yes, 2009
In the kidney, progesterone is inactivated to 20alpha-dihydro-progesterone (20alpha-DH-progesterone) to protect the mineralocorticoid receptor from progesterone excess.
Krozowski, Zygmunt   +6 more
core   +1 more source

On the substrate specificity of human CYP27A1

open access: yesJournal of Lipid Research, 2003
The mitochondrial sterol 27-hydroxylase (CYP27A1) is required for degradation of the C27-sterol side chain in bile acid biosynthesis. CYP27A1 seems, however, to have roles beyond this, as illustrated by patients with a deficient sterol 27-hydroxylase due
Maria Norlin   +3 more
doaj   +1 more source

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