نتایج جستجو برای: ژن als

تعداد نتایج: 42849  

Journal: :Journal of neurology, neurosurgery, and psychiatry 2007
J C Schymick Y Yang P M Andersen J P Vonsattel M Greenway P Momeni J Elder A Chiò G Restagno W Robberecht C Dahlberg O Mukherjee A Goate N Graff-Radford R J Caselli M Hutton J Gass A Cannon R Rademakers A B Singleton O Hardiman J Rothstein J Hardy B J Traynor

OBJECTIVE Mutations in the progranulin (PGRN) gene were recently described as the cause of ubiquitin positive frontotemporal dementia (FTD). Clinical and pathological overlap between amyotrophic lateral sclerosis (ALS) and FTD prompted us to screen PGRN in patients with ALS and ALS-FTD. METHODS The PGRN gene was sequenced in 272 cases of sporadic ALS, 40 cases of familial ALS and in 49 patien...

2013
William T. Hu Matthew Shelnutt Ashley Wilson Nicole Yarab Crystal Kelly Murray Grossman David J. Libon Jaffar Khan James J. Lah Allan I. Levey Jonathan Glass

BACKGROUND It is difficult to longitudinally characterize cognitive impairment in amyotrophic lateral sclerosis (ALS) due to motor deficits, and existing instruments aren't comparable with assessments in other dementias. METHODS The ALS Brief Cognitive Assessment (ALS-BCA) was validated in 70 subjects (37 with ALS) who also underwent detailed neuropsychological analysis. Cognitive predictors ...

Journal: :Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists 2012
Ioannis Zalonis Foteini Christidi Georgios Paraskevas Thomas Zabelis Ioannis Evdokimidis Evangelia Kararizou

Although executive functions in sporadic non-demented amyotrophic lateral sclerosis (ALS) patients are mostly affected, it remains unclear whether executive measures can differentiate between patients with bulbar and spinal ALS forms. Thirty spinal and 18 bulbar-onset ALS patients (ALS-s and ALS-b, respectively) as well as 47 demographically related healthy controls were examined in executive p...

2017
Yimin Mao Su-Wei Kuo Le Chen C J Heckman M C Jiang

Amyotrophic Lateral Sclerosis (ALS) is a devastative neurodegenerative disease characterized by selective loss of motoneurons. While several breakthroughs have been made in identifying ALS genetic defects, the detailed molecular mechanisms are still unclear. These genetic defects involve in numerous biological processes, which converge to a common destiny: motoneuron degeneration. In addition, ...

2017
Yuan-Yuan Shang Ming Yao Zhi-Wei Zhou Jian-Cui Li-Xia Rong-Ying Hu Ying-Yao Yu Qiong-Gao Biao-Yang Yu-Xi Liu Jie Dang Shu-Feng Zhou Nan-Yu

We investigated the efficacy of Alisertib (ALS), a selective Aurora kinase A (AURKA) inhibitor, in melanoma. We found that ALS exerts anti-proliferative, pro-apoptotic, and pro-autophagic effects on A375 and skmel-5 melanoma cells by inhibiting p38 MAPK signaling. SB202190, a p38 MAPK-selective inhibitor, enhanced ALS-induced apoptosis and autophagy in both cell lines. ALS induced cell cycle ar...

2013
Venkateswaran Rajagopalan Zao Liu Didier Allexandre Luduan Zhang Xiao-Feng Wang Erik P. Pioro Guang H. Yue

Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder. Current diagnosis time is about 12-months due to lack of objective methods. Previous brain white matter voxel based morphometry (VBM) studies in ALS reported inconsistent results. Fractal dimension (FD) has successfully been used to quantify brain WM shape complexity in various neurological disorders and agin...

Journal: :Genetics 2001
L L Hoyer R Fundyga J E Hecht J C Kapteyn F M Klis J Arnold

The ALS (agglutinin-like sequence) gene family of Candida albicans encodes cell-surface glycoproteins implicated in adhesion of the organism to host surfaces. Southern blot analysis with ALS-specific probes suggested the presence of ALS gene families in C. dubliniensis and C. tropicalis; three partial ALS genes were isolated from each organism. Northern blot analysis demonstrated that mechanism...

2013
Elise G. Valdés Svitlana Garbuzova-Davis

Trauma to the central nervous system (CNS) has been investigated as a risk factor for amyotrophic lateral sclerosis (ALS) despite conflicting epidemiological reports. Studies have suggested a link between ALS and traumatic axonal injury which complements the “dying back” theory of ALS. The theory suggests that neuronal dysfunction first occurs at the neuromuscular junction, and subsequent axona...

2010
Svitlana Garbuzova-Davis Robert L. Woods Michael K. Louis Theresa A. Zesiewicz Nicole Kuzmin-Nichols Kelly L. Sullivan Amber M. Miller Diana G. Hernandez-Ontiveros Paul R. Sanberg

BACKGROUND Amyotrophic Lateral Sclerosis (ALS) treatment is complicated by the various mechanisms underlying motor neuron degeneration. Recent studies showed that the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB) are compromised in an animal model of ALS due to endothelial cell degeneration. A later study demonstrated a loss of endothelium integrity in the spinal cords of ALS p...

2011
Fang Fang Honglei Chen Karin Wirdefeldt Lars-Olof Ronnevi Ammar Al-Chalabi Tracy L. Peters Freya Kamel Weimin Ye

BACKGROUND Severe infections may lead to chronic inflammation in the central nervous system (CNS) which may in turn play a role in the etiopathogenesis of amyotrophic lateral sclerosis (ALS). The relentless progression and invasive supportive treatments of ALS may on the other hand induce severe infections among ALS patients. METHODOLOGY AND PRINCIPAL FINDINGS The present study included 4,004...

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