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trans-1,4-bis(2-chlorobenzaminomethyl)cyclohexane dihydrochloride and Smith-Lemli-Opitz Syndrome

trans-1,4-bis(2-chlorobenzaminomethyl)cyclohexane dihydrochloride has been researched along with Smith-Lemli-Opitz Syndrome in 24 studies

Research

Studies (24)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's9 (37.50)18.2507
2000's9 (37.50)29.6817
2010's6 (25.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Fliesler, SJ; Keiko, U; Más Gómez, N; Mitchell, CH; Pfeffer, BA; Ramachandra Rao, S; Rowsam, AM; Skelton, LA; Sparrow, JR1
Ferrington, DA; Fliesler, SJ; Kapphahn, RJ; Richards, MJ1
Fliesler, SJ; Xu, L1
Fliesler, SJ; Liu, W; Porter, NA; Sheflin, LG; Xu, L1
Fliesler, SJ; Porter, NA; Sheflin, LG; Xu, L1
Fliesler, SJ; Goulah, CC; Keller, RK; Mitchell, DA1
Arnold, TP; Fliesler, SJ; Keller, RK1
Fliesler, SJ; Nagel, BA; Peachey, NS; Richards, MJ; Vaughan, DK1
Buchan, B; Fliesler, SJ; Peachey, NS; Richards, MJ; Vaughan, DK1
Fliesler, SJ; Jenewein, EC; Nagel, BA; Peachey, NS; Richards, MJ; Vaughan, DK1
Anderson, RE; Brush, RS; Fliesler, SJ; Ford, DA; Monda, JK; Richards, MJ1
Anderson, RE; Boesze-Battaglia, K; Brush, RS; Damek-Poprawa, M; Fliesler, SJ; Greeley, L; Mitchell, DC; Richards, MJ1
Chattopadhyay, A; Murty, MR; Paila, YD; Vairamani, M1
Chevy, F; Citadelle, D; Kolf-Clauw, M; Mulliez, N; Pham, J; Roux, C; Wolf, C1
Chevy, F; Citadelle, D; Kolf-Clauw, M; Roux, C; Siliart, B; Wolf, C1
Chambers, CM; McLean, MP; Ness, GC1
Batta, AK; Salen, G1
Chevy, F; Kolf-Clauw, M; Ponsart, C1
Clayton, PT1
Chevy, F; Gaoua, W; Roux, C; Wolf, C1
Fliesler, SJ; Miller, C; Peachey, NS; Richards, MJ1
Clotman, F; Gofflot, F; Kolf-Clauw, M; Picard, JJ; Roux, C1
Chevy, F; Gaoua, W; Ilien, F; Roux, C; Wolf, C1
Chevy, F; Citadelle, D; Cormier, V; Gaoua, W; Mulliez, N; Roux, C; Wolf, C1

Reviews

4 review(s) available for trans-1,4-bis(2-chlorobenzaminomethyl)cyclohexane dihydrochloride and Smith-Lemli-Opitz Syndrome

ArticleYear
Oxysterols and Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome: Implications for an Improved Therapeutic Intervention.
    Molecules (Basel, Switzerland), 2018, Oct-22, Volume: 23, Issue:10

    Topics: Animals; Cholesterol; Dehydrocholesterols; Disease Models, Animal; Lipid Metabolism; Oxysterols; Rats; Retina; Retinal Degeneration; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2018
Abnormal cholesterol biosynthesis produced by AY 9944 in the rat leads to skeletal deformities similar to the Smith-Lemli-Opitz syndrome.
    The Journal of laboratory and clinical medicine, 1998, Volume: 131, Issue:3

    Topics: Abnormalities, Drug-Induced; Animals; Anticholesteremic Agents; Bone and Bones; Bone Development; Cholesterol; Enzyme Inhibitors; Female; Holoprosencephaly; Humans; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pregnancy; Rats; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1998
Disorders of cholesterol biosynthesis.
    Archives of disease in childhood, 1998, Volume: 78, Issue:2

    Topics: Anticholesteremic Agents; Cholesterol; Desmosterol; Disease Models, Animal; Humans; Infant, Newborn; Lanosterol; Metabolism, Inborn Errors; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1998
Role of cholesterol in embryonic development.
    The American journal of clinical nutrition, 2000, Volume: 71, Issue:5 Suppl

    Topics: Animals; Anticholesteremic Agents; Cholesterol; Dehydrocholesterols; Disease Models, Animal; Embryonic and Fetal Development; Fetus; Piperazines; Rats; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride; Triparanol

2000

Other Studies

20 other study(ies) available for trans-1,4-bis(2-chlorobenzaminomethyl)cyclohexane dihydrochloride and Smith-Lemli-Opitz Syndrome

ArticleYear
Compromised phagosome maturation underlies RPE pathology in cell culture and whole animal models of Smith-Lemli-Opitz Syndrome.
    Autophagy, 2018, Volume: 14, Issue:10

    Topics: Animals; Biomarkers; Cathepsin D; Cattle; Cell Culture Techniques; Dehydrocholesterols; Disease Models, Animal; Humans; Lysosomes; Membrane Fusion; Phagocytosis; Phagosomes; Protein Biosynthesis; Rats; Retinal Pigment Epithelium; Retinoids; Rod Cell Outer Segment; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride; Transcription, Genetic; Ubiquitinated Proteins

2018
Lipid-derived and other oxidative modifications of retinal proteins in a rat model of Smith-Lemli-Opitz syndrome.
    Experimental eye research, 2019, Volume: 178

    Topics: Aldehydes; Animals; Blotting, Western; Disease Models, Animal; Electrophoresis, Polyacrylamide Gel; Enzyme Inhibitors; Female; Opsins; Oxidative Stress; Pregnancy; Proteomics; Rats; Rats, Sprague-Dawley; Retina; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride; Transducin

2019
Novel oxysterols observed in tissues and fluids of AY9944-treated rats: a model for Smith-Lemli-Opitz syndrome.
    Journal of lipid research, 2011, Volume: 52, Issue:10

    Topics: Animals; Anticholesteremic Agents; Cholestenones; Cholesterol; Chromatography, High Pressure Liquid; Dehydrocholesterols; Disease Models, Animal; Hydroxycholesterols; Ketocholesterols; Lipid Peroxidation; Magnetic Resonance Spectroscopy; Mass Spectrometry; Mice; Rats; Rats, Sprague-Dawley; Smith-Lemli-Opitz Syndrome; Spectrophotometry, Ultraviolet; Sterols; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2011
7-Dehydrocholesterol-derived oxysterols and retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome.
    Biochimica et biophysica acta, 2012, Volume: 1821, Issue:6

    Topics: Animals; Animals, Newborn; Cholesterol; Chromatography, High Pressure Liquid; Dehydrocholesterols; Disease Models, Animal; Female; Humans; Ketocholesterols; Male; Mass Spectrometry; Molecular Structure; Pregnancy; Rats; Rats, Sprague-Dawley; Retina; Retinal Degeneration; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2012
Hepatic isoprenoid metabolism in a rat model of Smith-Lemli-Opitz Syndrome.
    Lipids, 2013, Volume: 48, Issue:3

    Topics: Animals; Anticholesteremic Agents; Cholesterol; Dehydrocholesterols; Disease Models, Animal; Dolichols; Humans; Liver; Oxidoreductases Acting on CH-CH Group Donors; Rats; Rats, Sprague-Dawley; Smith-Lemli-Opitz Syndrome; Terpenes; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride; Ubiquinone

2013
Formation of 7-dehydrocholesterol-containing membrane rafts in vitro and in vivo, with relevance to the Smith-Lemli-Opitz syndrome.
    Journal of lipid research, 2004, Volume: 45, Issue:2

    Topics: Animals; Anticholesteremic Agents; Brain; Cattle; Cell Membrane; Cerebrosides; Dehydrocholesterols; Diet; Injections, Subcutaneous; Liposomes; Membrane Microdomains; Models, Chemical; Phosphatidylcholines; Rats; Smith-Lemli-Opitz Syndrome; Sphingomyelins; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2004
Retinal degeneration in a rodent model of Smith-Lemli-Opitz syndrome: electrophysiologic, biochemical, and morphologic features.
    Archives of ophthalmology (Chicago, Ill. : 1960), 2004, Volume: 122, Issue:8

    Topics: Animals; Anticholesteremic Agents; Cholesterol; Dark Adaptation; Dehydrocholesterols; Disease Models, Animal; Electrophysiology; Electroretinography; Female; Pigment Epithelium of Eye; Pregnancy; Rats; Rats, Sprague-Dawley; Retina; Retinal Degeneration; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2004
Light-induced exacerbation of retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome.
    Experimental eye research, 2006, Volume: 82, Issue:3

    Topics: Animals; Biomarkers; Cholesterol; Dark Adaptation; Docosahexaenoic Acids; Electroretinography; Free Radical Scavengers; Male; Models, Animal; Photic Stimulation; Photoreceptor Cells; Rats; Rats, Sprague-Dawley; Retina; Retinal Degeneration; Smith-Lemli-Opitz Syndrome; Thiourea; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2006
Partial rescue of retinal function and sterol steady-state in a rat model of Smith-Lemli-Opitz syndrome.
    Pediatric research, 2007, Volume: 61, Issue:3

    Topics: Animals; Cholesterol, Dietary; Disease Models, Animal; Female; Humans; Photoreceptor Cells, Vertebrate; Pregnancy; Rats; Retina; Smith-Lemli-Opitz Syndrome; Sterols; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2007
Lipidomic analysis of the retina in a rat model of Smith-Lemli-Opitz syndrome: alterations in docosahexaenoic acid content of phospholipid molecular species.
    Journal of neurochemistry, 2008, Volume: 105, Issue:3

    Topics: Animals; Cholesterol; Disease Models, Animal; Docosahexaenoic Acids; Neurochemistry; Neurons; Phosphatidylcholines; Phosphatidylethanolamines; Phospholipids; Rats; Retina; Retinal Degeneration; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2008
Alteration of retinal rod outer segment membrane fluidity in a rat model of Smith-Lemli-Opitz syndrome.
    Journal of lipid research, 2008, Volume: 49, Issue:7

    Topics: Animals; Disease Models, Animal; Fatty Acids; Female; Proteins; Rats; Rats, Sprague-Dawley; Reactive Oxygen Species; Rod Cell Outer Segment; Smith-Lemli-Opitz Syndrome; Sterols; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2008
Signaling by the human serotonin(1A) receptor is impaired in cellular model of Smith-Lemli-Opitz Syndrome.
    Biochimica et biophysica acta, 2008, Volume: 1778, Issue:6

    Topics: Animals; Anticholesteremic Agents; CHO Cells; Cricetinae; Cricetulus; Dehydrocholesterols; GTP-Binding Proteins; Humans; Ligands; Models, Biological; Protein Binding; Receptor, Serotonin, 5-HT1A; Signal Transduction; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2008
Changes in serum sterols of rats treated with 7-dehydrocholesterol-delta 7-reductase inhibitors: comparison to levels in humans with Smith-Lemli-Opitz syndrome.
    Journal of lipid research, 1996, Volume: 37, Issue:6

    Topics: Animals; Child; Cholestadienols; Cholesterol; Dehydrocholesterols; Disease Models, Animal; Enzyme Inhibitors; Female; Heterozygote; Humans; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Piperazines; Pregnancy; Prenatal Exposure Delayed Effects; Rats; Rats, Wistar; Smith-Lemli-Opitz Syndrome; Sterols; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1996
Inhibition of 7-dehydrocholesterol reductase by the teratogen AY9944: a rat model for Smith-Lemli-Opitz syndrome.
    Teratology, 1996, Volume: 54, Issue:3

    Topics: Animals; Cholesterol; Disease Models, Animal; Estradiol; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Progesterone; Rats; Rats, Wistar; Smith-Lemli-Opitz Syndrome; Teratogens; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1996
Smith-Lemli-Opitz syndrome produced in rats with AY 9944 treated by intravenous injection of lipoprotein cholesterol.
    American journal of medical genetics, 1997, Jan-31, Volume: 68, Issue:3

    Topics: Animals; Anticholesteremic Agents; Cholesterol; Cholesterol, Dietary; Combined Modality Therapy; Dehydrocholesterols; Disease Models, Animal; Enzyme Inhibitors; Injections, Intravenous; Lipoproteins; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Rats; Rats, Sprague-Dawley; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1997
Abnormal cholesterol biosynthesis as in Smith-Lemli-Opitz syndrome disrupts normal skeletal development in the rat.
    The Journal of laboratory and clinical medicine, 1998, Volume: 131, Issue:3

    Topics: Abnormalities, Drug-Induced; Animals; Anticholesteremic Agents; Body Weight; Bone and Bones; Bone Development; Cervical Vertebrae; Cholesterol; Dehydrocholesterols; Enzyme Inhibitors; Female; Gas Chromatography-Mass Spectrometry; Male; Osteogenesis; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pregnancy; Rats; Rats, Wistar; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1998
Oxidized derivatives of 7-dehydrocholesterol induce growth retardation in cultured rat embryos: a model for antenatal growth retardation in the Smith-Lemli-Opitz syndrome.
    Journal of lipid research, 1999, Volume: 40, Issue:3

    Topics: Animals; Antioxidants; Cholesterol; Dehydrocholesterols; Disease Models, Animal; Embryo, Mammalian; Embryonic and Fetal Development; Female; Growth; Organ Culture Techniques; Oxidation-Reduction; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Rats; Rats, Wistar; Smith-Lemli-Opitz Syndrome; Teratogens; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride; Ultraviolet Rays; Vitamin E

1999
Marked alteration of sterol metabolism and composition without compromising retinal development or function.
    Investigative ophthalmology & visual science, 1999, Volume: 40, Issue:8

    Topics: Animals; Animals, Newborn; Anticholesteremic Agents; Cholesterol; Chromatography, High Pressure Liquid; Dehydrocholesterols; Electroretinography; Female; Pregnancy; Rats; Rats, Sprague-Dawley; Retina; Smith-Lemli-Opitz Syndrome; Sterols; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

1999
Absence of ventral cell populations in the developing brain in a rat model of the Smith-Lemli-Opitz syndrome.
    American journal of medical genetics, 1999, Nov-26, Volume: 87, Issue:3

    Topics: Animals; Brain; Cholesterol; Disease Models, Animal; DNA-Binding Proteins; Embryonic and Fetal Development; Enzyme Inhibitors; Eye Proteins; Female; Fetal Diseases; Fetal Proteins; Gestational Age; Hedgehog Proteins; Homeodomain Proteins; In Situ Hybridization; Male; Nerve Tissue Proteins; Neural Tube Defects; Neurons; Otx Transcription Factors; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Paired Box Transcription Factors; PAX6 Transcription Factor; Pregnancy; Prosencephalon; Protein Biosynthesis; Proteins; Rats; Rats, Wistar; Repressor Proteins; Rhombencephalon; Signal Transduction; Smith-Lemli-Opitz Syndrome; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride; Trans-Activators

1999
Cholesterol deficit but not accumulation of aberrant sterols is the major cause of the teratogenic activity in the Smith-Lemli-Opitz syndrome animal model.
    Journal of lipid research, 2000, Volume: 41, Issue:4

    Topics: Abnormalities, Drug-Induced; Animals; Cholesterol; Cholesterol, Dietary; Dehydrocholesterols; Disease Models, Animal; Embryo, Mammalian; Female; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pregnancy; Rats; Rats, Wistar; Smith-Lemli-Opitz Syndrome; Steroid Isomerases; trans-1,4-Bis(2-chlorobenzaminomethyl)cyclohexane Dihydrochloride

2000