
NCBI . NLM . NIH . GOV {
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Title[redir]:
Bile acid biosynthesis in Smith-Lemli-Opitz syndrome bypassing cholesterol: Potential importance of pathway intermediates - PMC
Description:
New pathway of bile acid biosynthesis avoiding cholesterol. Mechanism for biosynthesis of 3β,7β-dihydroxy-5-ene bile acids. Biosynthesis of 3β-hydroxy-7-oxo-5-ene bile acids. Pathway intermediates bind to and activate Smo. A new link between SLOS ...
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Keywords {🔍}
doi, bile, acid, google, scholar, slos, pubmed, cholesterol, acids, pathway, fig, hoc, smo, pmc, free, article, dhc, signalling, plasma, crd, found, biosynthesis, urine, patients, human, fluid, βhydroxy, glcnac, metabolites, levels, oxysterols, samples, amniotic, binding, hedgehog, elevated, oxo, lipid, ββdihδba, liver, analysis, supplemental, βdihc, βhoca, data, receptor, npc, res, griffiths, sterol,
Topics {✒️}
urinary 3beta-sulfooxy-7beta-n-acetylglucosaminyl-5-cholen-24-oic acid 3α-hydroxy-5β-hydrogen configuration found 3beta-hydroxy-delta5-c27-steroid dehydrogenase 7β-dih-δ5-ba singly conjugated n-methyl-d-aspartate receptors n-methyl-d-aspartate receptor rsh/smith-lemli-opitz syndrome 7β-dih-δ5-ba define 7β-dih-δ5-ba glcnac conjugates smith-lemli-opitz syndrome patients 3β-hydroxy-7-oxo-5-ene bile acids 3β-hydroxy-δ5-c27-steroid oxidoreductase 7alpha-hydroxy-3-oxo-4-cholestenoic acid 3β-hydroxy-7-oxochol-5-en-24-oic exploiting charge-tagging utilising 7β-dihydroxy-5-ene bile acids 7β-dih-δ5-ba conjugated lc–ms exploiting charge-tagging smith-lemli-opitz syndrome gas chromatography/mass spectrometry 7α-hydroxy-3-oxo-4-ene structure 7β-hydroxy-3-oxo-4-ene structure charge-tagging mass spectrometry amino acid n-acyltransferase 7β-dihydroxychol-5-enoic acids 7β-hydroxy-3-oxo-4-ene sterols peroxisomal side-chain shortening force-field approaches incorporated lc/esi-ms/ms analysis 7α-dihydroxy-5-ene structure tri-hydroxylated cholanoic acids 7β-dih-δ5-ba 7β-dih-δ5-ba [72] high-performance liquid chromatography 7β-dihydroxy-5-ene structure 8-carbon side-chain attached pmc beta search bile acyl coa-synthetase 7α-hydroxy-5-ene sterols niemann-pick disease type 7β-dihydroxy-5-ene substrates δ5-unsaturated glcnac conjugates pmc copyright notice 24-trihydroxycholest-5-en-26-oic acid 25-trihydroxycholest-5-en-26-oic acid 7β-dihydroxy-5-ene sterols alpha-methylacyl-coa-racemase structure-based druggability predictions 7β-dihydroxy-5-ene function grant numbers bb/i001735/1
Questions {❓}
- 27-Hydroxycholesterol, does it exist?
- Cerebrospinal fluid steroidomics: are bioactive bile acids present in brain?
- Holoprosencephaly in RSH/Smith-Lemli-Opitz syndrome: does abnormal cholesterol metabolism affect the function of Sonic Hedgehog?
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- https://doi.org/10.1007/s00018-003-3476-y's financial summary
- What's the profit of https://scholar.google.com/scholar_lookup?journal=Cell.%20Mol.%20Life%20Sci.&title=Human%20and%20rodent%20type%201%2011beta-hydroxysteroid%20dehydrogenases%20are%207beta-hydroxycholesterol%20dehydrogenases%20involved%20in%20oxysterol%20metabolism&author=M.%20Hult&author=B.%20Elleby&author=N.%20Shafqat&author=S.%20Svensson&author=A.%20Rane&volume=61&publication_year=2004&pages=992-999&pmid=15095019&doi=10.1007/s00018-003-3476-y&?
- How much does https://doi.org/10.1016/j.freeradbiomed.2007.04.033 generate monthly?
- How much does https://scholar.google.com/scholar_lookup?journal=Free%20Radic.%20Biol.%20Med.&title=In%20vivo%20interconversion%20of%207beta-hydroxycholesterol%20and%207-ketocholesterol,%20potential%20surrogate%20markers%20for%20oxidative%20stress&author=H.%20Larsson&author=Y.%20Bottiger&author=L.%20Iuliano&author=U.%20Diczfalusy&volume=43&publication_year=2007&pages=695-701&pmid=17664133&doi=10.1016/j.freeradbiomed.2007.04.033& net monthly?
- Revenue of https://doi.org/10.1074/jbc.M313615200
- How much does https://scholar.google.com/scholar_lookup?journal=J.%20Biol.%20Chem.&title=Rapid%20hepatic%20metabolism%20of%207-ketocholesterol%20by%2011beta-hydroxysteroid%20dehydrogenase%20type%201:%20species-specific%20differences%20between%20the%20rat,%20human,%20and%20hamster%20enzyme&author=R.A.%20Schweizer&author=M.%20Zurcher&author=Z.%20Balazs&author=B.%20Dick&author=A.%20Odermatt&volume=279&publication_year=2004&pages=18415-18424&pmid=14973125&doi=10.1074/jbc.M313615200& rake in every month?
- How much cash flow does https://doi.org/10.1373/clinchem.2014.231332 have monthly?
- How much does https://scholar.google.com/scholar_lookup?journal=Clin.%20Chem.&title=Quantitative%20charge-tags%20for%20sterol%20and%20oxysterol%20analysis&author=P.J.%20Crick&author=T.%20William%20Bentley&author=J.%20Abdel-Khalik&author=I.%20Matthews&author=P.T.%20Clayton&volume=61&publication_year=2015&pages=400-411&pmid=25512642&doi=10.1373/clinchem.2014.231332& bring in each month?
- https://doi.org/10.1194/jlr.P071639's revenue stream
- How much money does https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=Defective%20cholesterol%20metabolism%20in%20amyotrophic%20lateral%20sclerosis&author=J.%20Abdel-Khalik&author=E.%20Yutuc&author=P.J.%20Crick&author=J.A.%20Gustafsson&author=M.%20Warner&volume=58&publication_year=2017&pages=267-278&pmid=27811233&doi=10.1194/jlr.P071639& make?
- https://doi.org/10.1016/j.jsbmb.2016.03.018's revenue stream
- How much does https://scholar.google.com/scholar_lookup?journal=J.%20Steroid%20Biochem.%20Mol.%20Biol.&title=Sterols%20and%20oxysterols%20in%20plasma%20from%20Smith-Lemli-Opitz%20syndrome%20patients&author=W.J.%20Griffiths&author=J.%20Abdel-Khalik&author=P.J.%20Crick&author=M.%20Ogundare&author=C.H.%20Shackleton&volume=169&publication_year=2017&pages=77-87&pmid=26976653&doi=10.1016/j.jsbmb.2016.03.018& pull in monthly?
- What's the revenue for https://doi.org/10.1038/sj.jp.7211290?
- Earnings of https://scholar.google.com/scholar_lookup?journal=J.%20Perinatol.&title=Amniotic%20fluid:%20not%20just%20fetal%20urine%20anymore&author=M.A.%20Underwood&author=W.M.%20Gilbert&author=M.P.%20Sherman&volume=25&publication_year=2005&pages=341-348&pmid=15861199&doi=10.1038/sj.jp.7211290&
- How much profit is https://doi.org/10.1016/0009-8981(95)06038-4 making per month?
- What's the total monthly financial gain of https://scholar.google.com/scholar_lookup?journal=Clin.%20Chim.%20Acta&title=Diagnosis%20of%20Smith-Lemli-Opitz%20syndrome%20by%20gas%20chromatography/mass%20spectrometry%20of%207-dehydrocholesterol%20in%20plasma,%20amniotic%20fluid%20and%20cultured%20skin%20fibroblasts&author=R.I.%20Kelley&volume=236&publication_year=1995&pages=45-58&pmid=7664465&doi=10.1016/0009-8981(95)06038-4&?
- https://doi.org/10.1373/clinchem.2007.100644's financial summary
- What's the financial outcome of https://scholar.google.com/scholar_lookup?journal=Clin.%20Chem.&title=Potential%20of%20sterol%20analysis%20by%20liquid%20chromatography-tandem%20mass%20spectrometry%20for%20the%20prenatal%20diagnosis%20of%20Smith-Lemli-Opitz%20syndrome&author=W.J.%20Griffiths&author=Y.%20Wang&author=K.%20Karu&author=E.%20Samuel&author=S.%20McDonnell&volume=54&publication_year=2008&pages=1317-1324&pmid=18556335&doi=10.1373/clinchem.2007.100644&?
- Learn how profitable https://doi.org/10.1074/jbc.271.34.20903 is on a monthly basis
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- How much income is https://doi.org/10.1002/hep.1840200412 earning monthly?
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- Profit of https://doi.org/10.1038/nchembio.765
- Get to know https://scholar.google.com/scholar_lookup?journal=Nat.%20Chem.%20Biol.&title=Oxysterols%20are%20allosteric%20activators%20of%20the%20oncoprotein%20Smoothened&author=S.%20Nachtergaele&author=L.K.%20Mydock&author=K.%20Krishnan&author=J.%20Rammohan&author=P.H.%20Schlesinger&volume=8&publication_year=2012&pages=211-220&pmid=22231273&doi=10.1038/nchembio.765&'s earnings
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- How much does https://scholar.google.com/scholar_lookup?journal=Nature&title=Structural%20basis%20of%20Smoothened%20regulation%20by%20its%20extracellular%20domains&author=E.F.%20Byrne&author=R.%20Sircar&author=P.S.%20Miller&author=G.%20Hedger&author=G.%20Luchetti&volume=535&publication_year=2016&pages=517-522&pmid=27437577&doi=10.1038/nature18934& generate monthly?
- What's the financial intake of https://doi.org/10.1002/jcc.21334?
- Financial intake of https://scholar.google.com/scholar_lookup?journal=J.%20Comput.%20Chem.&title=AutoDock%20Vina:%20improving%20the%20speed%20and%20accuracy%20of%20docking%20with%20a%20new%20scoring%20function,%20efficient%20optimization,%20and%20multithreading&author=O.%20Trott&author=A.J.%20Olson&volume=31&publication_year=2010&pages=455-461&pmid=19499576&doi=10.1002/jcc.21334&
- What's the financial gain of https://doi.org/10.7554/eLife.20304?
- How much does https://scholar.google.com/scholar_lookup?journal=Elife&title=Cholesterol%20activates%20the%20G-protein%20coupled%20receptor%20smoothened%20to%20promote%20Hedgehog%20signaling&author=G.%20Luchetti&author=R.%20Sircar&author=J.H.%20Kong&author=S.%20Nachtergaele&author=A.%20Sagner&publication_year=2016&pages=5&pmid=27705744&doi=10.7554/eLife.20304& make?
- Discover the revenue of https://doi.org/10.1007/s11745-001-0775-8
- What's the income of https://scholar.google.com/scholar_lookup?journal=Lipids&title=Metabolism%20of%20an%20oxysterol,%207-ketocholesterol,%20by%20sterol%2027-hydroxylase%20in%20HepG2%20cells&author=M.A.%20Lyons&author=A.J.%20Brown&volume=36&publication_year=2001&pages=701-711&pmid=11521968&doi=10.1007/s11745-001-0775-8&?
- How much profit does https://doi.org/10.1194/jlr.M092908 make?
- How much profit does https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=11beta-Hydroxysteroid%20dehydrogenases%20control%20access%20of%207beta,27-dihydroxycholesterol%20to%20retinoid-related%20orphan%20receptor%20gamma&author=K.R.%20Beck&author=S.G.%20Inderbinen&author=S.%20Kanagaratnam&author=D.V.%20Kratschmar&author=A.M.%20Jetten&volume=60&publication_year=2019&pages=1535-1546&pmid=31273032&doi=10.1194/jlr.M092908& make?
- How much cash flow does https://doi.org/10.1194/jlr.M014084 have monthly?
- Learn about the earnings of https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=Cholesterol%2025-hydroxylation%20activity%20of%20CYP3A&author=A.%20Honda&author=T.%20Miyazaki&author=T.%20Ikegami&author=J.%20Iwamoto&author=T.%20Maeda&volume=52&publication_year=2011&pages=1509-1516&pmid=21576599&doi=10.1194/jlr.M014084&
- What's the profit of https://doi.org/10.1016/j.jsbmb.2019.03.011?
- How much profit does https://scholar.google.com/scholar_lookup?journal=J.%20Steroid%20Biochem.%20Mol.%20Biol.&title=Enzymatic%20interconversion%20of%20the%20oxysterols%207beta,25-dihydroxycholesterol%20and%207-keto,25-hydroxycholesterol%20by%2011beta-hydroxysteroid%20dehydrogenase%20type%201%20and%202&author=K.R.%20Beck&author=S.%20Kanagaratnam&author=D.V.%20Kratschmar&author=J.%20Birk&author=H.%20Yamaguchi&volume=190&publication_year=2019&pages=19-28&pmid=30902677&doi=10.1016/j.jsbmb.2019.03.011& generate?
- How much does https://doi.org/10.1002/(SICI)1096-8628(19961230)66:4<478::AID-AJMG22>3.0.CO;2-Q pull in?
- What are the earnings of https://scholar.google.com/scholar_lookup?journal=Am.%20J.%20Med.%20Genet.&title=Holoprosencephaly%20in%20RSH/Smith-Lemli-Opitz%20syndrome:%20does%20abnormal%20cholesterol%20metabolism%20affect%20the%20function%20of%20Sonic%20Hedgehog?&author=R.L.%20Kelley&author=E.%20Roessler&author=R.C.%20Hennekam&author=G.L.%20Feldman&author=K.%20Kosaki&volume=66&publication_year=1996&pages=478-484&pmid=8989473&doi=10.1002/(SICI)1096-8628(19961230)66:4<478::AID-AJMG22>3.0.CO;2-Q&?
- Revenue of https://doi.org/10.1172/JCI2962
- What's the revenue for https://scholar.google.com/scholar_lookup?journal=J.%20Clin.%20Invest.&title=Identification%20of%20a%20new%20inborn%20error%20in%20bile%20acid%20synthesis:%20mutation%20of%20the%20oxysterol%207alpha-hydroxylase%20gene%20causes%20severe%20neonatal%20liver%20disease&author=K.D.%20Setchell&author=M.%20Schwarz&author=N.C.%20O%E2%80%99Connell&author=E.G.%20Lund&author=D.L.%20Davis&volume=102&publication_year=1998&pages=1690-1703&pmid=9802883&doi=10.1172/JCI2962&?
- How profitable is https://doi.org/10.1203/00006450-198701000-00021?
- How much income is https://scholar.google.com/scholar_lookup?journal=Pediatr.%20Res.&title=Bile%20acid%20metabolism%20during%20development:%20metabolism%20of%20lithocholic%20acid%20in%20human%20fetal%20liver&author=J.%20Gustafsson&author=S.%20Anderson&author=J.%20Sjovall&volume=21&publication_year=1987&pages=99-103&pmid=3797138&doi=10.1203/00006450-198701000-00021& earning monthly?
- What is the monthly revenue of https://doi.org/10.1159/000242087?
- Revenue of https://scholar.google.com/scholar_lookup?journal=Neonatology&title=Bile%20acid%20metabolism%20during%20development:%20metabolism%20of%20taurodeoxycholic%20acid%20in%20human%20fetal%20liver&author=J.%20Gustafsson&author=S.%20Andersson&author=J.%20Sj%C3%B6vall&volume=47&publication_year=1985&pages=26-31&pmid=3967055&doi=10.1159/000242087&
- https://doi.org/10.1093/database/baw030's revenue stream
- What is the monthly revenue of https://scholar.google.com/scholar_lookup?journal=Database%20(Oxford)&title=Genic%20insights%20from%20integrated%20human%20proteomics%20in%20GeneCards&author=S.%20Fishilevich&author=S.%20Zimmerman&author=A.%20Kohn&author=T.%20Iny%20Stein&author=T.%20Olender&publication_year=2016&pages=2016&pmid=27048349&doi=10.1093/database/baw030&?
- How much revenue does https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=Bile%20acid%20biosynthesis%20during%20development:%20hydroxylation%20of%20C27-sterols%20in%20human%20fetal%20liver&author=J.%20Gustafsson&volume=27&publication_year=1986&pages=801-806&pmid=3772248& bring in?
- What's the income generated by https://doi.org/10.1194/jlr.M014217 each month?
- How much revenue does https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=Conversion%20of%207-ketocholesterol%20to%20oxysterol%20metabolites%20by%20recombinant%20CYP27A1%20and%20retinal%20pigment%20epithelial%20cells&author=G.Y.%20Heo&author=I.%20Bederman&author=N.%20Mast&author=W.L.%20Liao&author=I.V.%20Turko&volume=52&publication_year=2011&pages=1117-1127&pmid=21411718&doi=10.1194/jlr.M014217& produce monthly?
- https://doi.org/10.1016/j.psyneuen.2016.04.006's financial summary
- How much money does https://scholar.google.com/scholar_lookup?journal=Psychoneuroendocrinology&title=Tandem%20mass%20spectrometry%20determined%20maternal%20cortisone%20to%20cortisol%20ratio%20and%20psychiatric%20morbidity%20during%20pregnancy-interaction%20with%20birth%20weight&author=C.%20Hellgren&author=A.%20Edvinsson&author=J.D.%20Olivier&author=R.%20Fornes&author=E.%20Stener-Victorin&volume=69&publication_year=2016&pages=142-149&pmid=27088373&doi=10.1016/j.psyneuen.2016.04.006& make?
- What's the monthly income of https://scholar.google.com/scholar_lookup?journal=J.%20Biol.%20Chem.&title=Hepatic%20bile%20acid%20metabolism%20during%20early%20development%20revealed%20from%20the%20analysis%20of%20human%20fetal%20gallbladder%20bile&author=K.D.%20Setchell&author=R.%20Dumaswala&author=C.%20Colombo&author=M.%20Ronchi&volume=263&publication_year=1988&pages=16637-16644&pmid=3182806&?
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- Learn how profitable https://scholar.google.com/scholar_lookup?journal=Clin.%20Chim.%20Acta&title=Profile%20of%20bile%20acids%20in%20fetal%20gallbladder%20and%20meconium%20using%20liquid%20chromatography-tandem%20mass%20spectrometry&author=N.%20Naritaka&author=M.%20Suzuki&author=H.%20Sato&author=H.%20Takei&author=T.%20Murai&volume=446&publication_year=2015&pages=76-81&pmid=25887994&doi=10.1016/j.cca.2015.04.008& is on a monthly basis
- How much money does https://doi.org/10.1002/ajmg.1320500413 make?
- Profit of https://scholar.google.com/scholar_lookup?journal=Am.%20J.%20Med.%20Genet.&title=Abnormal%20bile%20acids%20in%20the%20Smith-Lemli-Opitz%20syndrome&author=M.R.%20Natowicz&author=J.E.%20Evans&volume=50&publication_year=1994&pages=364-367&pmid=8209917&doi=10.1002/ajmg.1320500413&
- Earnings of https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=Bile%20acid%20synthesis%20in%20the%20Smith-Lemli-Opitz%20syndrome:%20effects%20of%20dehydrocholesterols%20on%20cholesterol%207alpha-hydroxylase%20and%2027-hydroxylase%20activities%20in%20rat%20liver&author=A.%20Honda&author=G.%20Salen&author=S.%20Shefer&author=A.K.%20Batta&author=M.%20Honda&volume=40&publication_year=1999&pages=1520-1528&pmid=10428990&
- What's the monthly money flow for https://scholar.google.com/scholar_lookup?journal=J.%20Lipid%20Res.&title=Sterol%20balance%20in%20the%20Smith-Lemli-Opitz%20syndrome.%20Reduction%20in%20whole%20body%20cholesterol%20synthesis%20and%20normal%20bile%20acid%20production&author=R.D.%20Steiner&author=L.M.%20Linck&author=D.P.%20Flavell&author=D.S.%20Lin&author=W.E.%20Connor&volume=41&publication_year=2000&pages=1437-1447&pmid=10974051&?
- https://scholar.google.com/scholar_lookup?journal=Hepatology&title=Formation%20of%207%20alpha-%20and%207%20beta-hydroxylated%20bile%20acid%20precursors%20from%2027-hydroxycholesterol%20in%20human%20liver%20microsomes%20and%20mitochondria&author=J.%20Shoda&author=A.%20Toll&author=M.%20Axelson&author=F.%20Pieper&author=K.%20Wikvall&volume=17&publication_year=1993&pages=395-403&pmid=8444412&'s financial summary
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- How much revenue does https://doi.org/10.1242/dev.02393 bring in?
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- Check the income stats for https://doi.org/10.1038/s41586-019-1355-4
- Financial intake of https://scholar.google.com/scholar_lookup?journal=Nature&title=Smoothened%20stimulation%20by%20membrane%20sterols%20drives%20Hedgehog%20pathway%20activity&author=I.%20Deshpande&author=J.%20Liang&author=D.%20Hedeen&author=K.J.%20Roberts&author=Y.%20Zhang&volume=571&publication_year=2019&pages=284-288&pmid=31263273&doi=10.1038/s41586-019-1355-4&
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- Income figures for https://scholar.google.com/scholar_lookup?journal=Science&title=Patched1%20regulates%20hedgehog%20signaling%20at%20the%20primary%20cilium&author=R.%20Rohatgi&author=L.%20Milenkovic&author=M.P.%20Scott&volume=317&publication_year=2007&pages=372-376&pmid=17641202&doi=10.1126/science.1139740&
- What's the total monthly financial gain of https://doi.org/10.1038/nchembio.1290?
- How much cash flow does https://scholar.google.com/scholar_lookup?journal=Nat.%20Chem.%20Biol.&title=Oxysterol%20binding%20to%20the%20extracellular%20domain%20of%20Smoothened%20in%20Hedgehog%20signaling&author=D.%20Nedelcu&author=J.%20Liu&author=Y.%20Xu&author=C.%20Jao&author=A.%20Salic&volume=9&publication_year=2013&pages=557-564&pmid=23831757&doi=10.1038/nchembio.1290& have monthly?
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