Results 51 to 60 of about 9,389 (199)

Alternative pre-mRNA splicing of the sterol 27-hydroxylase gene (CYP 27) caused by a G to A mutation at the last nucleotide of exon 6 in a patient with cerebrotendinous xanthomatosis (CTX)

Journal of Lipid Research, 1998
A recently identified G to A mutation at the last nucleotide of exon 6 of the sterol 27-hydroxylase gene (CYP 27) in a patient with cerebrotendinous xanthomatosis (CTX) was shown here to cause alternative pre-mRNA splicing of the gene.
Wengen Chen, Shunichiro Kubota, Yousuke Seyama   +2 more
doaj   +1 more source

Effect of increasing the expression of cholesterol transporters (StAR, MLN64, and SCP-2) on bile acid synthesis

Journal of Lipid Research, 2004
There are two major pathways of bile acid synthesis: the “neutral” pathway, initiated by highly regulated microsomal cholesterol 7α-hydroxylase (CYP7A1), and an “alternative” pathway, initiated by mitochondrial sterol 27-hydroxylase (CYP27A1).
Shunlin Ren, Phillip Hylemon, Dalila Marques, Elizabeth Hall, Kaye Redford, Gregorio Gil, William M. Pandak   +6 more
doaj   +1 more source

Branched‐Chain Amino and Keto Acids Reduce Hepatocyte Lipid Droplet Size and Number via Distinct Proteomic Pathways

PROTEOMICS, EarlyView.
ABSTRACT Branched‐chain amino acids (BCAA) and their corresponding keto acids (BCKA) have been associated with changes in hepatic lipid metabolism and the resulting alterations in intracellular triglyceride concentrations. In this study, we utilized a previously established hepatocyte model to investigate the impact of BCAA and BCKA supplementation on ...
Jayasimha R. Daddam, Mounica Sura, Edward Kolodziej, Maria Gojcaj, Zheng Zhou   +4 more
wiley   +1 more source

The Cdc42 effectors Ste20, Cla4, and Skm1 down-regulate the expression of genes involved in sterol uptake by a mitogen-activated protein kinase-independent pathway [PDF]

, 2009
© 2009 by The American Society for Cell Biology. Under the License and Publishing Agreement, authors grant to the general public, effective two months after publication of (i.e.,.
Jacquier, N, Höfken, T, Lin, Meng, Jacquier, Nicolas, Schneiter, R, Unden, H, Just, Ursula, Just, U, Unden, Heike, Höfken, Thomas, Schneiter, Roger   +10 more
core   +1 more source

Phenotypic characterization of Lith genes that determine susceptibility to cholesterol cholelithiasis in inbred mice: integrated activities of hepatic lipid regulatory enzymes1

Journal of Lipid Research, 1999
There is no consensus whether hepatic lipid regulatory enzymes play primary or secondary roles in cholesterol cholelithiasis. We have used inbred mice with Lith genes that determine cholesterol gallstone susceptibility to evaluate the question.
Frank Lammert, David Q-H. Wang, Beverly Paigen, Martin C. Carey   +3 more
doaj   +1 more source

Steroidogenic compensation and lipid deficiency with enhanced NAD+ salvage in small‐for‐gestational‐age placenta

The FEBS Journal, EarlyView.
Fetal growth restriction is associated with placental metabolic adaptations. In small‐for‐gestational‐age placenta (SGA), cholesterol receptors and steroidogenic enzymes are upregulated, enhancing steroidogenesis. NAD salvage pathway is also increased to support NADP+/NADPH requirements.
Serena Xodo, Lorenza Driul, Vanessa Tolotto, Eros Di Giorgio, Luigi E. Xodo   +4 more
wiley   +1 more source

Cerebrotendinous xanthomatosis: report of two Brazilian brothers Xantomatose cerebrotendínea: relato de dois irmãos brasileiros

Arquivos de Neuro-Psiquiatria, 2004
Cerebrotendinous xanthomatosis is a treatable rare autossomal recessive disease characterized by lipid storage secondary to a sterol 27-hydroxylase deficiency in the formation of cholic and chenodeoxycholic acids.
Marcos Christiano Lange, Viviane Flumignan Zétola, Helio A.G. Teive, Rosana H. Scola, Ana Paula Trentin, Jorge A. Zavala, Eduardo R. Pereira, Salmo Raskin, Lineu C. Werneck, Erik A. Sistermans   +9 more
doaj   +1 more source

Downregulation of Cyp7a1 by Cholic Acid and Chenodeoxycholic Acid in Cyp27a1/ApoE Double Knockout Mice: Differential Cardiovascular Outcome

Frontiers in Endocrinology, 2020
Sterol 27-hydroxylase (CYP27A1) is a key enzyme in bile acids (BAs) biosynthesis and a regulator of cholesterol metabolism. Cyp27a1/Apolipoprotein E double knockout (DKO) mice fed with western diet (WD) are protected from atherosclerosis via up ...
Line Zurkinden, Line Zurkinden, Line Zurkinden, Dmitri Sviridov, Dmitri Sviridov, Bruno Vogt, Bruno Vogt, Genevieve Escher, Genevieve Escher   +8 more
doaj   +1 more source

Therapeutic potential of okra (Abelmoschus esculentus) in dysglycaemia and metabolic dysfunction: A systematic review and meta‐analysis across the diabetes spectrum

Experimental Physiology, EarlyView.
Abstract The aim of this systematic review and meta‐analysis was to evaluate comprehensively the therapeutic potential of Abelmoschus esculentus (okra) supplementation across the diabetes spectrum of key metabolic risk factors. A search was conducted in PubMed, Scopus, Web of Science, EMBASE and the Cochrane Library, up to 23 July 2025, to identify ...
Ali Jafari, Helia Mardani, Mohammad Amin Karimi, MohammadHossein Sahami Gilan, Helia Hemmat, Fatemeh Shoja, Nooshin Enayati Soofi, Ghazaleh Eslamian   +7 more
wiley   +1 more source

A novel Tetrahymena thermophila sterol C-22 desaturase belongs to the fatty acid hydroxylase/desaturase superfamily [PDF]

, 2022
Sterols in eukaryotic cells play important roles in modulating membrane fluidity and in cell signaling and trafficking. During evolution, a combination of gene losses and acquisitions gave rise to an extraordinary diversity of sterols in different ...
García Siburu, Nicolás Pablo, Gargiulo, Laura Belén, Maldonado, Lucas L., Nusblat, Alejandro David, Siburu, Nicolás G., Maldonado, Lucas Luciano, Gargiulo, Laura B., Sanchez Granel, María L., Nudel, Clara B., Nusblat, Alejandro D., Uttaro, Antonio D., Sánchez Granel, María Luz, Fricska, Annamária, Nudel, Berta Clara, Uttaro, Antonio Domingo   +14 more
core   +1 more source