Literature DB >> 29033641

Do Low Serum UCH-L1 and TDP-43 Levels Indicate Disturbed Ubiquitin-Proteosome System in Autism Spectrum Disorder?

İhsan Çetin1, İhsan Tezdiğ2, Mahmut Cem Tarakçioğlu3, Muhammed Tayyib Kadak4, Ömer Faruk Demirel5, Ömer Faruk Özer6, Fırat Erdoğan7, Burak Doğangün4.   

Abstract

INTRODUCTION: The mechanism of ubiquitination-related abnormalities causing neural development problems is still unclear. We examined the association between autism and serum transactive response DNA-binding protein-43 (TDP-43) and ubiquitin c-terminal hydrolase-L1 (UCH-L1) levels, both of which are members of the ubiquitin-proteosome system.
METHODS: We measured serum levels of TDP-43 and UCH-L1 in 24 children with autism and 24 healthy children. Childhood Autism Rating Scale (CARS) was used to assess symptom severity at admission.
RESULTS: The mean serum TDP-43 and UCH-L1 levels in children with autism spectrum disorder (ASD) were found to decrease compared to healthy controls (p<0.001, 506.21±780.97 ng/L and 1245.80±996.76 ng/L, respectively; 3.08±5.44 ng/mL and 8.64±6.67 ng/mL, respectively). A positive correlation between serum TDP-43 levels and UCH-L1 levels was found in the ASD group (r=0.947, n=24, p<0.001). The CARS score of children with ASD was 48.91 points (standard deviation [SD]: 5.82).
CONCLUSION: Low serum levels of UCH-L1 and TDP-43 may reflect disturbed ubiquitination in autism.

Entities:  

Keywords:  Autism; TDP-43; UCH-L1; ubiquitination

Year:  2017        PMID: 29033641      PMCID: PMC5630107          DOI: 10.5152/npa.2017.14873

Source DB:  PubMed          Journal:  Noro Psikiyatr Ars        ISSN: 1300-0667            Impact factor:   1.339


  45 in total

1.  Loss of mTOR-dependent macroautophagy causes autistic-like synaptic pruning deficits.

Authors:  Guomei Tang; Kathryn Gudsnuk; Sheng-Han Kuo; Marisa L Cotrina; Gorazd Rosoklija; Alexander Sosunov; Mark S Sonders; Ellen Kanter; Candace Castagna; Ai Yamamoto; Zhenyu Yue; Ottavio Arancio; Bradley S Peterson; Frances Champagne; Andrew J Dwork; James Goldman; David Sulzer
Journal:  Neuron       Date:  2014-08-21       Impact factor: 17.173

2.  TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein.

Authors:  Michael J Strong; Kathryn Volkening; Robert Hammond; Wencheng Yang; Wendy Strong; Cheryl Leystra-Lantz; Christen Shoesmith
Journal:  Mol Cell Neurosci       Date:  2007-03-20       Impact factor: 4.314

3.  Reduction in memory in passive avoidance learning, exploratory behaviour and synaptic plasticity in mice with a spontaneous deletion in the ubiquitin C-terminal hydrolase L1 gene.

Authors:  Mikako Sakurai; Masayuki Sekiguchi; Ko Zushida; Kazuyuki Yamada; Satoshi Nagamine; Tomohiro Kabuta; Keiji Wada
Journal:  Eur J Neurosci       Date:  2008-02       Impact factor: 3.386

4.  Oxidative modifications and down-regulation of ubiquitin carboxyl-terminal hydrolase L1 associated with idiopathic Parkinson's and Alzheimer's diseases.

Authors:  Joungil Choi; Allan I Levey; Susan T Weintraub; Howard D Rees; Marla Gearing; Lih-Shen Chin; Lian Li
Journal:  J Biol Chem       Date:  2004-01-13       Impact factor: 5.157

5.  Autism genome-wide copy number variation reveals ubiquitin and neuronal genes.

Authors:  Joseph T Glessner; Kai Wang; Guiqing Cai; Olena Korvatska; Cecilia E Kim; Shawn Wood; Haitao Zhang; Annette Estes; Camille W Brune; Jonathan P Bradfield; Marcin Imielinski; Edward C Frackelton; Jennifer Reichert; Emily L Crawford; Jeffrey Munson; Patrick M A Sleiman; Rosetta Chiavacci; Kiran Annaiah; Kelly Thomas; Cuiping Hou; Wendy Glaberson; James Flory; Frederick Otieno; Maria Garris; Latha Soorya; Lambertus Klei; Joseph Piven; Kacie J Meyer; Evdokia Anagnostou; Takeshi Sakurai; Rachel M Game; Danielle S Rudd; Danielle Zurawiecki; Christopher J McDougle; Lea K Davis; Judith Miller; David J Posey; Shana Michaels; Alexander Kolevzon; Jeremy M Silverman; Raphael Bernier; Susan E Levy; Robert T Schultz; Geraldine Dawson; Thomas Owley; William M McMahon; Thomas H Wassink; John A Sweeney; John I Nurnberger; Hilary Coon; James S Sutcliffe; Nancy J Minshew; Struan F A Grant; Maja Bucan; Edwin H Cook; Joseph D Buxbaum; Bernie Devlin; Gerard D Schellenberg; Hakon Hakonarson
Journal:  Nature       Date:  2009-04-28       Impact factor: 49.962

6.  Chronological requirements of TDP-43 function in synaptic organization and locomotive control.

Authors:  Giulia Romano; Raffaella Klima; Emanuele Buratti; Patrik Verstreken; Francisco E Baralle; Fabian Feiguin
Journal:  Neurobiol Dis       Date:  2014-08-01       Impact factor: 5.996

7.  TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease.

Authors:  Catalina Amador-Ortiz; Wen-Lang Lin; Zeshan Ahmed; David Personett; Peter Davies; Ranjan Duara; Neill R Graff-Radford; Michael L Hutton; Dennis W Dickson
Journal:  Ann Neurol       Date:  2007-05       Impact factor: 10.422

8.  Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease.

Authors:  Claudia Schwab; Tetsuaki Arai; Masato Hasegawa; Sheng Yu; Patrick L McGeer
Journal:  J Neuropathol Exp Neurol       Date:  2008-12       Impact factor: 3.685

Review 9.  The neurobiology of autism spectrum disorders.

Authors:  M Parellada; M J Penzol; L Pina; C Moreno; E González-Vioque; G Zalsman; C Arango
Journal:  Eur Psychiatry       Date:  2013-11-22       Impact factor: 5.361

10.  Ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) is increased in cerebrospinal fluid and plasma of patients after epileptic seizure.

Authors:  Stefania Mondello; Johanna Palmio; Jackson Streeter; Ronald L Hayes; Jukka Peltola; Andreas Jeromin
Journal:  BMC Neurol       Date:  2012-08-29       Impact factor: 2.474

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Authors:  Ankur Rakesh Dubey; Som Mohanlal Patwa; Sumit Kinger; Yuvraj Anandrao Jagtap; Prashant Kumar; Sarika Singh; Rohan Dhiman; Hem Chandra Jha; Amit Mishra
Journal:  Mol Neurobiol       Date:  2022-03-19       Impact factor: 5.682

2.  Clinical features of behavioral symptoms in patients with semantic dementia: Does semantic dementia cause autistic traits?

Authors:  Shizuka Sakuta; Mamoru Hashimoto; Manabu Ikeda; Asuka Koyama; Akihiro Takasaki; Maki Hotta; Ryuji Fukuhara; Tomohisa Ishikawa; Seiji Yuki; Yusuke Miyagawa; Yosuke Hidaka; Keiichiro Kaneda; Minoru Takebayashi
Journal:  PLoS One       Date:  2021-02-18       Impact factor: 3.240

3.  Urinary proteome profiling for children with autism using data-independent acquisition proteomics.

Authors:  Wenshu Meng; Yuhang Huan; Youhe Gao
Journal:  Transl Pediatr       Date:  2021-07
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