Successful treatment of infantile oxysterol 7α-hydroxylase deficiency with oral chenodeoxycholic acid [PDF]
BMC Gastroenterology, 2021 Background Deficiency of oxysterol 7α-hydroxylase, encoded by CYP7B1, is associated with fatal infantile progressive intrahepatic cholestasis and hereditary spastic paraplegia type 5.
Yun-Ping Tang +5 more
doaj +4 more sources
Structure and functions of human oxysterol 7α-hydroxylase cDNAs and gene CYP7B1
Journal of Lipid Research, 1999 Oxysterol 7α-hydroxylase has broad substrate specificity for sterol metabolites and may be involved in many metabolic processes including bile acid synthesis and neurosteroid metabolism.
John Y L Chiang
exaly +4 more sources
Targeting Brain Cholesterol Homeostasis in Alzheimer's Disease: Mechanisms and Therapeutic Perspectives. [PDF]
J NeurochemBrain cholesterol homeostasis is tightly regulated and largely independent of peripheral cholesterol. This review proposes a unifying framework in which aging, sex, and related biological stressors, including hypercholesterolemia, neurodegeneration, inflammation, and oxidative stress, converge to disrupt cholesterol homeostasis in the brain ...
Ruthirakuhan M, Taha AY, Swardfager W.
europepmc +2 more sources
Cholesterol Reprograms Oxysterol Metabolism via the LOX1/CH25H/CYP7B1 Signaling Axis to Drive Multidrug Resistance in Colorectal Cancer. [PDF]
Hum MutatChemotherapeutic resistance remains a major contributor to tumor recurrence and unfavorable clinical outcomes in colorectal cancer (CRC). Although cholesterol metabolic reprogramming has been implicated in tumorigenesis, metastasis, and drug resistance across multiple malignancies, its specific role in CRC chemoresistance requires systematic ...
Cheng H +6 more
europepmc +2 more sources
Cells, 2021 Ambient temperature is an important determinant of both the alternative bile acid synthesis pathway controlled by oxysterol 7-α hydroxylase (CYP7B1) and the progression of metabolic-associated fatty liver disease (MAFLD).
Ioannis Evangelakos +2 more
exaly +3 more sources
Treatment of Inborn Errors by Product Replacement: The Example of Inborn Errors of Bile Acid Synthesis. [PDF]
J Inherit Metab DisABSTRACT Many inborn errors of metabolism affect pathways involved in the synthesis of a metabolite that has an important biochemical or physiological function, and adverse effects of the disorder can be attributed to the lack of this metabolite. Thus, there is the opportunity for treatment by ‘product replacement’. One of the disorders in the pathways
Clayton PT, Hirachan R, Murphy E.
europepmc +2 more sources
Complete Recovery of Oxysterol 7α-Hydroxylase Deficiency by Living Donor Transplantation in a 4-Month-Old Infant: the First Korean Case Report and Literature Review. [PDF]
J Korean Med Sci, 2018 Hong J +5 more
europepmc +2 more sources
Liver specific transgenic expression of CYP7B1 attenuates early western diet-induced MASLD progression [PDF]
Journal of Lipid ResearchEffect of liver specific oxysterol 7α-hydroxylase (CYP7B1) overexpression on the Western diet (WD)-induced metabolic dysfunction-associated steatotic liver disease (MASLD) progression was studied in mice. Among various hepatic genes impacted during MASLD
Genta Kakiyama +13 more
doaj +2 more sources
Oxysterol 7α-Hydroxylase Activity by Cholesterol 7α-Hydroxylase (CYP7A) [PDF]
Journal of Biological Chemistry, 2000 Maria Norlin +2 more
exaly +2 more sources
Disruption of the Oxysterol 7α-Hydroxylase Gene in Mice [PDF]
Journal of Biological Chemistry, 2000 Erik G Lund +2 more
exaly +2 more sources