دورية أكاديمية
أعرض في EDS

Effect of post-mortem delay on N-terminal huntingtin protein fragments in human control and Huntington disease brain lysates.

التفاصيل البيبلوغرافية
العنوان: Effect of post-mortem delay on N-terminal huntingtin protein fragments in human control and Huntington disease brain lysates.
المؤلفون: Schut MH; Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands., Patassini S; Centre for Brain Research and Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand., Kim EH; Centre for Brain Research and Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand., Bullock J; Centre for Brain Research and Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand., Waldvogel HJ; Centre for Brain Research and Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand., Faull RLM; Centre for Brain Research and Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand., Pepers BA; Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands., den Dunnen JT; Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.; Leiden Genome Technology Center, Leiden University Medical Center, Leiden, The Netherlands., van Ommen GB; Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands., van Roon-Mom WMC; Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.
المصدر: PloS one [PLoS One] 2017 Jun 01; Vol. 12 (6), pp. e0178556. Date of Electronic Publication: 2017 Jun 01 (Print Publication: 2017).
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Public Library of Science Country of Publication: United States NLM ID: 101285081 Publication Model: eCollection Cited Medium: Internet ISSN: 1932-6203 (Electronic) Linking ISSN: 19326203 NLM ISO Abbreviation: PLoS One Subsets: MEDLINE
أسماء مطبوعة: Original Publication: San Francisco, CA : Public Library of Science
مواضيع طبية MeSH: Brain/*metabolism , Huntingtin Protein/*metabolism , Huntington Disease/*pathology, 3' Untranslated Regions ; Aged ; Aged, 80 and over ; Amino Acid Sequence ; Brain/pathology ; Case-Control Studies ; Cell Line ; Female ; HEK293 Cells ; Humans ; Huntington Disease/metabolism ; Male ; Middle Aged ; Open Reading Frames ; Postmortem Changes ; Sequence Homology, Amino Acid
مستخلص: Huntington disease is associated with elongation of a CAG repeat in the HTT gene that results in a mutant huntingtin protein. Several studies have implicated N-terminal huntingtin protein fragments in Huntington disease pathogenesis. Ideally, these fragments are studied in human brain tissue. However, the use of human brain tissue comes with certain unavoidable variables such as post mortem delay, artefacts from freeze-thaw cycles and subject-to-subject variation. Knowledge on how these variables might affect N-terminal huntingtin protein fragments in post mortem human brain is important for a proper interpretation of study results. The effect of post mortem delay on protein in human brain is known to vary depending on the protein of interest. In the present study, we have assessed the effect of post mortem delay on N-terminal huntingtin protein fragments using western blot. We mimicked post mortem delay in one individual control case and one individual Huntington disease case with low initial post mortem delay. The influence of subject-to-subject variation on N-terminal huntingtin fragments was assessed in human cortex and human striatum using two cohorts of control and Huntington disease subjects. Our results show that effects of post mortem delay on N-terminal huntingtin protein fragments are minor in our individual subjects. Additionally, one freeze-thaw cycle decreases the huntingtin western blot signal intensity in the cortex control subject, but does not introduce additional N-terminal huntingtin fragments. Our results suggest that subject-to-subject variation contributes more to variability in N-terminal huntingtin fragments than post mortem delay.
References: J Neurosci. 2002 Sep 15;22(18):7862-72. (PMID: 12223539)
PLoS One. 2016 Mar 16;11(3):e0151615. (PMID: 26982086)
Neuron. 1995 Nov;15(5):1193-201. (PMID: 7576661)
Brain Res. 1990 May 28;517(1-2):269-75. (PMID: 1695864)
Exp Toxicol Pathol. 2004 Dec;56(3):159-71. (PMID: 15625785)
Hum Mol Genet. 2003 Jul 1;12 (13):1555-67. (PMID: 12812983)
Cell Tissue Bank. 2012 Dec;13(4):597-606. (PMID: 21922206)
Cell. 1996 Nov 1;87(3):493-506. (PMID: 8898202)
J Biol Chem. 2004 May 7;279(19):20211-20. (PMID: 14981075)
Hum Mol Genet. 2015 Feb 15;24(4):913-25. (PMID: 25305076)
J Neurochem. 2002 Jul;82(1):84-92. (PMID: 12091468)
Brain Res Bull. 2007 Apr 30;72 (2-3):83-97. (PMID: 17352931)
J Neurosci Res. 2009 Apr;87(5):1156-61. (PMID: 19006085)
Neurol Sci. 2015 Mar;36(3):429-34. (PMID: 25294428)
J Neurosci Methods. 2008 Mar 30;169(1):135-40. (PMID: 18201768)
Cell. 1993 Mar 26;72(6):971-83. (PMID: 8458085)
J Neuropathol Exp Neurol. 1985 Nov;44(6):559-77. (PMID: 2932539)
Mol Cell. 2002 Aug;10 (2):259-69. (PMID: 12191472)
J Neurosci. 2012 Jan 4;32(1):183-93. (PMID: 22219281)
J Biol Chem. 2000 Jun 30;275(26):19831-8. (PMID: 10770929)
Nucleic Acid Ther. 2014 Feb;24(1):4-12. (PMID: 24380395)
J Neurosci. 2001 Mar 15;21(6):1830-7. (PMID: 11245667)
EMBO J. 2015 Sep 2;34(17 ):2255-71. (PMID: 26165689)
Cell. 2006 Jun 16;125(6):1179-91. (PMID: 16777606)
J Biol Chem. 2015 Jul 31;290(31):19287-306. (PMID: 26025364)
Cell Tissue Bank. 2008 Sep;9(3):169-79. (PMID: 18357514)
Science. 1995 Jul 21;269(5222):407-10. (PMID: 7618107)
Biochem Biophys Res Commun. 2015 Jan 2;456(1):66-73. (PMID: 25446099)
J Neurosci Methods. 2004 Oct 30;139(2):153-9. (PMID: 15488227)
Cell Tissue Bank. 2008 Sep;9(3):181-94. (PMID: 18543077)
Nat Biotechnol. 2009 May;27(5):478-84. (PMID: 19412185)
Brain Res. 1993 Dec 3;629(2):225-30. (PMID: 8111626)
Forensic Sci Int. 1996 Aug 15;81(2-3):165-74. (PMID: 8837492)
المشرفين على المادة: 0 (3' Untranslated Regions)
0 (Huntingtin Protein)
تواريخ الأحداث: Date Created: 20170602 Date Completed: 20170926 Latest Revision: 20181113
رمز التحديث: 20240829
مُعرف محوري في PubMed: PMC5453542
DOI: 10.1371/journal.pone.0178556
PMID: 28570578
قاعدة البيانات: MEDLINE
الوصف
تدمد:1932-6203
DOI:10.1371/journal.pone.0178556