title: Altered expression of 3-betahydroxysterol delta-24-reductase/selective Alzheimer's disease indicator-1 gene in Huntington's disease models.
abstract: BACKGROUND
  3-betahydroxysterol delta-24-reductase (DHCR24), also called selective Alzheimer's disease indicator-1, is a crucial enzyme in cholesterol biosynthesis with neuroprotective properties that is downregulated in brain areas affected by Alzheimer's disease.
  
  
  OBJECTIVE
  In the present study, we investigated modifications of DHCR24 expression in models of Huntington's disease (HD), a neurodegenerative disorder caused by a polyglutamine expansion in huntingtin (Htt) protein that induces degeneration of cerebral cortex and striatum as well as lateral hypothalamic abnormality.
  
  
  METHODS
  Basal expression of DHCR24 and its modulation after oxidative stress were evaluated in rat striatal precursors cells (ST14A) transfected with wild-type (Htt) or mutant Htt (mHtt) and in brain tissue of an HD mouse model (R6/2).
  
  
  RESULTS
  The results showed that DHCR24 transcript levels were decreased in ST14A cells expressing mHtt and in the brain of symptomatic R6/2 mice, but were significantly increased in ST14A cells overexpressing wild-type Htt. In addition, we demonstrated that, in the striatal precursors, the decrease of DHCR24 expression in response to oxidative stress was modified according to the presence of Htt or of its mutant form. Preliminary results indicated a modification of DHCR24 expression in post-mortem brain samples of HD patients.
  
  
  CONCLUSIONS
  In conclusion, these results support the hypothesis of a possible role of DHCR24 in HD.
authors.affiliation: Department of Pharmacological and Biomolecular Sciences, Section of Biomedicine and Endocrinology, and Centre of Excellence on Neurodegenerative Diseases (CEND), Università degli Studi di Milano, Via Balzaretti, 9, 20133, Milan, Italy.|University of Oslo and Norwegian Center for Stem Cell Research, 0317, Oslo, Norway.
authors.forename: Athina
authors.initials: A
authors.lastname: Samara
authors.affiliation: Department of Pharmacological and Biomolecular Sciences, Section of Biomedicine and Endocrinology, and Centre of Excellence on Neurodegenerative Diseases (CEND), Università degli Studi di Milano, Via Balzaretti, 9, 20133, Milan, Italy.
authors.forename: Mariarita
authors.initials: M
authors.lastname: Galbiati
authors.affiliation: Endocrine Unit, Department of Clinical Physiopathology, Center for Research, Transfer and High Education on Chronic, Inflammatory, Degenerative and Neoplastic Disorders for the Development of Novel Therapies' (DENOThe), University of Florence, Viale Pieraccini, 6, 50139, Florence, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Luciani
authors.affiliation: Endocrine Unit, Department of Clinical Physiopathology, Center for Research, Transfer and High Education on Chronic, Inflammatory, Degenerative and Neoplastic Disorders for the Development of Novel Therapies' (DENOThe), University of Florence, Viale Pieraccini, 6, 50139, Florence, Italy.
authors.forename: Cristiana
authors.initials: C
authors.lastname: Deledda
authors.affiliation: Department of Pharmacological and Biomolecular Sciences, Section of Biomedicine and Endocrinology, and Centre of Excellence on Neurodegenerative Diseases (CEND), Università degli Studi di Milano, Via Balzaretti, 9, 20133, Milan, Italy.
authors.forename: Elio
authors.initials: E
authors.lastname: Messi
authors.affiliation: Endocrine Unit, Department of Clinical Physiopathology, Center for Research, Transfer and High Education on Chronic, Inflammatory, Degenerative and Neoplastic Disorders for the Development of Novel Therapies' (DENOThe), University of Florence, Viale Pieraccini, 6, 50139, Florence, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Peri
authors.affiliation: Department of Pharmacological and Biomolecular Sciences, Section of Biomedicine and Endocrinology, and Centre of Excellence on Neurodegenerative Diseases (CEND), Università degli Studi di Milano, Via Balzaretti, 9, 20133, Milan, Italy. roberto.maggi@unimi.it.
authors.forename: Roberto
authors.initials: R
authors.lastname: Maggi
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D001921:Brain / Q000201:enzymology* / Q000378:metabolism; D002460:Cell Line; D005260:Female; D015971:Gene Expression Regulation, Enzymologic; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000201:enzymology* / Q000378:metabolism; D008297:Male; D008822:Mice, Transgenic; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D009474:Neurons / Q000201:enzymology* / Q000378:metabolism; D018384:Oxidative Stress; D044925:Oxidoreductases Acting on CH-CH Group Donors / Q000235:genetics / Q000378:metabolism*; D010296:Parietal Lobe / Q000201:enzymology / Q000378:metabolism; D012333:RNA, Messenger / Q000378:metabolism; D051381:Rats; D011994:Recombinant Proteins / Q000378:metabolism; D018911:Trinucleotide Repeats
chemical_list: C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D012333:RNA, Messenger; D011994:Recombinant Proteins; D044925:Oxidoreductases Acting on CH-CH Group Donors; C437069:3beta-hydroxysterol delta24-reductase; C499055:DHCR24 protein, human; C499056:Dhcr24 protein, mouse