Research map: Baat ‐Deficient Mice Recapitulate Elevated 7α‐Hydroxy‐3‐Oxo‐4‐Cholestenoic Acid Observed in a Japanese Patient With BAAT Deficiency
Back to the article
Papers in this map
- The Enzymes, Regulation, and Genetics of Bile Acid Synthesis · David W. Russell · 2003 · 2080 citations · Cited by this paper
- Metabolism Controls the Timing of Human Brain Development and Maturation · 2026 · Related
- Fibroblast growth factor 15 functions as an enterohepatic signal to regulate bile acid homeostasis · Takeshi Inagaki · 2005 · 1860 citations · Cited by this paper
- Liver Gene Therapy in Fabry Disease Mice With Low Doses of rAAV2 /8 Expressing a Codon‐Optimized h GLA cDNA Results in Long‐Term Disease Correction · 2026 · Related
- Bile Acid Physiology · Agostino Di Ciaula · 2017 · 570 citations · Cited by this paper
- Vitamin‐Responsive Disorders: From Molecular Basis to Clinical Presentation and Therapy · 2026 · Related
- Protocol for Primary Mouse Hepatocyte Isolation · Meital Charni‐Natan · 2020 · 443 citations · Cited by this paper
- Quantitative Muscle MRI of the Lower Extremities Reveals Different Patterns of Involvement in Classic Infantile and Young Late‐Onset Pompe Patients · 2026 · Related
- Oxysterols: From cholesterol metabolites to key mediators · Valentin Mutemberezi · 2016 · 337 citations · Cited by this paper
- Tri‐Parametric Assessment of α‐Galactosidase A Activity, lysoGb3 and X‐Inactivation Aids Genotype‐Phenotype Categorization of Fabry Disease Female Patients · 2026 · Related
- Complex inheritance of familial hypercholanemia with associated mutations in TJP2 and BAAT · Victoria E. H. Carlton · 2003 · 332 citations · Cited by this paper
- Acute Neurological Complications After Transplantation in Methylmalonic Acidemia: A 35‐Patient French Cohort · 2026 · Related
- Up to date on cholesterol 7 alpha-hydroxylase (CYP7A1) in bile acid synthesis · John Y.L. Chiang · 2020 · 296 citations · Cited by this paper
- Maternal and Fetal Outcomes in Imiglucerase‐Treated Patients With Gaucher Disease: Real‐World Evidence From the International Collaborative Gaucher Group ( ICGG ) Gaucher Registry Pregnancy Sub‐Registry · 2026 · Related
- A Compact, High-Accuracy Cas9 with a Dinucleotide PAM for In Vivo Genome Editing · Alireza Edraki · 2018 · 282 citations · Cited by this paper
- How Much AI Can Scientific Publishing Tolerate? · 2026 · Related
- Animal models to study bile acid metabolism · Jianing Li · 2018 · 261 citations · Cited by this paper
- Mapping Clinical Progression to Brain Atrophy in CLN2 Patients Under Cerliponase Alfa Treatment: A Prospective Neuroimaging Study · 2026 · Related
- Bile acid metabolism and signalling in liver disease · Claudia Daniela Fuchs · 2024 · 225 citations · Cited by this paper
- Immune Dysregulation in Branched Chain Organic Acidemias · 2026 · Related
- Molecular Basis of Bile Acid-FXR-FGF15/19 Signaling Axis · Takeshi Katafuchi · 2022 · 189 citations · Cited by this paper
- The acidic pathway of bile acid synthesis: Not just an alternative pathway · William M. Pandak · 2019 · 177 citations · Cited by this paper
- Bile Acid Synthesis: From Nature to the Chemical Modification and Synthesis and Their Applications as Drugs and Nutrients · Tanja Šarenac · 2018 · 159 citations · Cited by this paper
- Levels of 7α‐hydroxy‐4‐cholesten‐3‐one in plasma reflect rates of bile acid synthesis in man · Magnus Axelson · 1988 · 147 citations · Cited by this paper
- Genetic Defects in Bile Acid Conjugation Cause Fat-Soluble Vitamin Deficiency · Kenneth D.R. Setchell · 2013 · 133 citations · Cited by this paper
- Oxysterol research: a brief review · William James Griffiths · 2019 · 107 citations · Cited by this paper
- Intestinal FXR-mediated FGF15 production contributes to diurnal control of hepatic bile acid synthesis in mice · Johanna H. M. Stroeve · 2010 · 100 citations · Cited by this paper
- Inborn Errors of Bile Acid Metabolism · James E. Heubi · 2018 · 82 citations · Cited by this paper
- Bile acid conjugation deficiency causes hypercholanemia, hyperphagia, islet dysfunction, and gut dysbiosis in mice · Bandar D. Alrehaili · 2022 · 32 citations · Cited by this paper
- Diagnosis in bile acid-CoA: amino acid N-acyltransferase deficiency. · Nedim Hadžić · 2012 · 31 citations · Cited by this paper
- Roles of nuclear receptors in the up-regulation of hepatic cholesterol 7α-hydroxylase by cholestyramine in rats · Shinya Shibata · 2006 · 28 citations · Cited by this paper
- Baat Gene Knockout Alters Post-Natal Development, the Gut Microbiome, and Reveals Unusual Bile Acids in Mice · Kerri A. Neugebauer · 2022 · 28 citations · Cited by this paper
- Comparison of simple extraction procedures in liquid chromatographymass spectrometry based determination of serum 7α-hydroxy-4-cholesten-3-one, a surrogate marker of bile acid synthesis · Martin LeníL·ek · 2016 · 23 citations · Cited by this paper
- Changes in conjugated urinary bile acids across age groups · Keiko Sato · 2020 · 19 citations · Cited by this paper
- Insulin dysregulation drives mitochondrial cholesterol metabolite accumulation: initiating hepatic toxicity in nonalcoholic fatty liver disease · Kei Minowa · 2023 · 17 citations · Cited by this paper
- Inborn errors of bile acid metabolism in Japan · Tatsuki Mizuochi · 2023 · 16 citations · Cited by this paper
- Bile Acid Metabolism · Paul A. Dawson · 2015 · 12 citations · Cited by this paper
- Abcb4-defect cholangitis mouse model with hydrophobic bile acid composition by in vivo liver-specific gene deletion · Kota Tsuruya · 2024 · 6 citations · Cited by this paper
- Urine bile acid profile of a Japanese patient with a bile acid amidation defect · Nakayuki Naritaka · 2022 · 5 citations · Cited by this paper
Source: OpenAlex (CC0)