搜索页_Familial amyotrophic lateral sclerosis (FALS)

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在“Elsevier电子期刊”中，命中：220条，耗时：0.015996 秒

在所有数据库中总计命中：97条

1.Replication study of MATR3 in familial and sporadic amyotrophic lateral sclerosis

作者：Claire S. Leblond^a ; ^b ; Ziv Gan-Or^a ; ^b ; Dan Spiegelman^b ; ^c ; Sandra B. Laurent^b ; ^c ; Anna Szuto^b ; ^c ; Alan Hodgkinson^d ; Alexandre Dionne-Laporte^b ; ^c ; Pierre Provencher^e ; Mamede de Carvalho^f ; Sandro Orrù ; ^g ; Denis Brunet^e ; ^h ; Jean-Pierre Bouchard^e ; ^h ; Philip Awadalla^d ; ⁱ ; Nicolas Dupré ; ^e ; ^h ; Patrick A. Dion^b ; ^c ; Guy A. Rouleau^b ; ^c ; ^{guy.rouleau@mcgill.ca" class="auth_mail" title="E-mail the corresponding author}

关键词：Amyotrophic lateral sclerosis ; Matrin-3 ; Nuclear matrix protein and DNA/RNA binding protein ; French-Canadian population ; Genetic mutation

刊名：Neurobiology of Aging

出版年：2016

2.cIAPs promote the proteasomal degradation of mutant SOD1 linked to familial amyotrophic lateral sclerosis

作者：Jin Sun Choi^a ; Kidae Kim^a ; ^d ; Do Hee Lee^g ; Sayeon Cho^h ; Jae Du Ha^f ; Byoung Chul Park^a ; ^d ; Sunhong Kim^a ; ^e ; ^{sunhong@kribb.re.kr" class="auth_mail" title="E-mail the corresponding author} ; Sung Goo Park^a ; ^c ; ^{sgpark@kribb.re.kr" class="auth_mail" title="E-mail the corresponding author} ; Jeong-Hoon Kim^b ; ^c ; ^{jhoonkim@kribb.re.kr" class="auth_mail" title="E-mail the corresponding author}

关键词：FALS ; SOD1 ; Ubiquitination ; cIAPs

刊名：Biochemical and Biophysical Research Communications

出版年：2016

3.Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis

作者：Xavier Gallart-Palau^a ; ^b ; ^c ; ^{xavgallart@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Chee-Hoe Ng^e ; ^f ; Joan Ribera^a ; ^b ; Siu Kwan Sze^c ; Kah-Leong Lim^d ; ^e ; ^f

关键词：FALS ; SOD1G93A ; Mitochondria ; Drosophila

刊名：Neuroscience Letters

出版年：2016

4.Two distinct clinical features and cognitive impairment in amyotrophic lateral sclerosis patients with TARDBP gene mutations in the Chinese population

作者：XiaoDong Ju^a ; ¹ ; WenChao Liu^b ; ¹ ; XiaoGang Li^c ; Na Liu^c ; Nan Zhang^c ; Tao Liu^b ; Min Deng^b ; ^{dengmin1706@gmail.com" class="auth_mail" title="E-mail the corresponding author}

关键词：Amyotrophic lateral sclerosis ; TARDBP gene mutation ; Clinical phenotype ; Genotype ; Cognitive impairment

刊名：Neurobiology of Aging

出版年：2016

5.SOD1 in neurotoxicity and its controversial roles in SOD1 mutation-negative ALS

作者：Yuki Hayashi ; Kengo Homma ; Hidenori Ichijo ; ^{ichijo@mol.f.u-tokyo.ac.jp" class="auth_mail" title="E-mail the corresponding author}

关键词：Amyotrophic lateral sclerosis (ALS) ; Familial amyotrophic lateral sclerosis (FALS) ; Sporadic amyotrophic lateral sclerosis (SALS) ; Cu ; Zn superoxide dismutase (SOD1) ; Neurodegeneration ; Motor neuron

刊名：Advances in Biological Regulation

出版年：2016

6.Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis

作者：Yulei Shang^a ; Eric J. Huang^a ; ^b ; ^{eric.huang2@ucsf.edu" class="auth_mail" title="E-mail the corresponding author}

关键词：RNA binding protein ; Fused in sarcoma (FUS) ; Prion-like property ; Low complexity domain ; DNA damage repair ; RNA splicing ; Amyotrophic lateral sclerosis (ALS) ; Frontotemporal dementia (FTD)

刊名：Brain Research

出版年：2016

7.Hydrogen peroxide-mediated induction of SOD1 gene transcription is independent from Nrf2 in a cellular model of neurodegeneration

关键词：ALS ; Amyotrophic Lateral Sclerosis ; ARE/EpRE ; Antioxidant/Electrophile responsive Element ; CEs ; Composite Elements ; fALS ; familial Amyotrophic Lateral Sclerosis ; NDs ; Neurodegenerative Disorders ; OS ; Oxidative stress ; PD ; Parkinson's disease ; PBMCs ; Peripheral Blood Mononuclear Cells ; ROS ; Reactive Oxygen Species ; sALS ; Sporadic Amyotrophic Lateral Sclerosis ; tBHQ ; tert-butylhydroquinone ; TF ; Transcription Factor ; TSS ; Transcription Start Site ; XRE ; Xenobiotic Responsive Element

刊名：Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms

出版年：2016

8.Preservation of neuromuscular function in symptomatic SOD1-G93A mice by peripheral infusion of methylene blue

作者：Janet D. Talbot^a ; John N. Barrett^a ; ^b ; Doris Nonner^a ; Zhongsheng Zhang^a ; Kyle Wicomb^a ; Ellen F. Barrett^a ; ^b ; ^{ebarrett2@med.miami.edu" class="auth_mail" title="E-mail the corresponding author}

关键词：ALS ; amyotrophic lateral sclerosis ; BgTx ; α-bungarotoxin ; EDL ; extensor digitorum longus muscle ; ETC ; electron transport chain ; fALS ; familial amyotrophic lateral sclerosis ; FF ; fast ; fatiguable muscle ; FUS ; fused in sarcoma/translated in liposarcoma ; MB ; methylene blue ; SOD1 ; superoxide dismutase 1 ; TA ; tibialis anterior muscle ; TDP-43 ; transactive response DNA-binding protein 43 ; YFP ; yellow fluorescent protein

刊名：Experimental Neurology

出版年：2016

9.Proteomic analysis of FUS interacting proteins provides insights into FUS function and its role in ALS

作者：Marisa Kamelgarn^a ; ^b ; ¹ ; ^{meka229@g.uky.edu" class="auth_mail" title="E-mail the corresponding author} ; Jing Chen^a ; ¹ ; ^{jchen4@email.uky.edu" class="auth_mail" title="E-mail the corresponding author} ; Lisha Kuang^a ; ^{lisha.kuang@uky.edu" class="auth_mail" title="E-mail the corresponding author} ; Alexandra Arenas^a ; ^b ; ^{maar223@g.uky.edu" class="auth_mail" title="E-mail the corresponding author} ; Jianjun Zhai^a ; ^{jianjunzhai@hotmail.com" class="auth_mail" title="E-mail the corresponding author} ; Haining Zhu^a ; ^b ; ^c ; ^{haining@uky.edu" class="auth_mail" title="E-mail the corresponding author} ; Jozsef Gal^a ; ^{jgal2@uky.edu" class="auth_mail" title="E-mail the corresponding author}

关键词：ALS ; amyotrophic lateral sclerosis ; fALS ; familial ALS ; sALS ; sporadic ALS ; 293T ; human embryonic kidney (HEK293T) cells ; FUS/TLS ; Fused in Sarcoma/Translocated in Liposarcoma ; GST ; glutathione S-transferase ; RIPA ; radioimmunoprecipitation assay buffer ; SDS-PAGE ; denaturing polyacrylamide gel electrophoresis ; TBST ; TRIS-buffered saline/Tween-20 buffer ; PBS ; phosphate-buffered saline buffer ; EGFP ; enhanced green fluorescent protein ; BSA ; bovine serum albumin ; DAPI ; 4&prime ; 6-diamidino-2-phenylin

刊名：Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

出版年：2016

10.Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem Cells

作者：Naoki Ichiyanagi¹ ; ⁸ ; Koki Fujimori¹ ; ⁸ ; Masato Yano¹ ; ² ; ^{myano@med.niigata-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Chikako Ishihara-Fujisaki¹ ; Takefumi Sone¹ ; Tetsuya Akiyama³ ; Yohei Okada¹ ; ⁴ ; Wado Akamatsu⁵ ; Takuya Matsumoto¹ ; Mitsuru Ishikawa¹ ; Yoshinori Nishimoto¹ ; Yasuharu Ishihara¹ ; Tetsushi Sakuma⁶ ; Takashi Yamamoto⁶ ; Hitomi Tsuiji⁷ ; Naoki Suzuki³ ; Hitoshi Warita³ ; Masashi Aoki³ ; Hideyuki Okano¹ ; ^{hidokano@a2.keio.jp" class="auth_mail" title="E-mail the corresponding author}

刊名：Stem Cell Reports

出版年：2016

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