نتایج جستجو برای: ncl

تعداد نتایج: 963  

2012
Fred E. Indig Ivana Rybanska Parimal Karmakar Chakravarty Devulapalli Haiqing Fu France Carrier Vilhelm A. Bohr

BACKGROUND The Werner protein (WRNp), a member of the RecQ helicase family, is strongly associated with the nucleolus, as is nucleolin (NCL), an important nucleolar constituent protein. Both WRNp and NCL respond to the effects of DNA damaging agents. Therefore, we have investigated if these nuclear proteins interact and if this interaction has a possible functional significance in DNA damage re...

Journal: :Behavioral neuroscience 2002
S Lissek B Diekamp O Güntürkün

The neostriatum caudolaterale (NCL) in the pigeon (Columba livia) forebrain is a multisensory associative area and a functional equivalent to the mammalian prefrontal cortex (PFC). To investigate the role of N-methyl-D-aspartate (NMDA) receptors in the NCL for learning flexibility, the authors trained pigeons in a color reversal task while locally blocking NMDA receptors with D,L-2-2-amino-5-ph...

2012
Jose Bras Alain Verloes Susanne A. Schneider Sara E. Mole Rita J. Guerreiro

Neuronal ceroid lipofuscinoses (NCLs) comprise a heterogeneous group of metabolic storage diseases that present with the accumulation of autofluorescent lipopigment, neurodegeneration and premature death. Nine genes have been thus far identified as the cause of different types of NCL, with ages at onset ranging from around birth to adult, although the underlying etiology of the disease still re...

Journal: :American journal of human genetics 2012
Katherine R Smith John Damiano Silvana Franceschetti Stirling Carpenter Laura Canafoglia Michela Morbin Giacomina Rossi Davide Pareyson Sara E Mole John F Staropoli Katherine B Sims Jada Lewis Wen-Lang Lin Dennis W Dickson Hans-Henrik Dahl Melanie Bahlo Samuel F Berkovic

We performed hypothesis-free linkage analysis and exome sequencing in a family with two siblings who had neuronal ceroid lipofuscinosis (NCL). Two linkage peaks with maximum LOD scores of 3.07 and 2.97 were found on chromosomes 7 and 17, respectively. Unexpectedly, we found these siblings to be homozygous for a c.813_816del (p.Thr272Serfs∗10) mutation in the progranulin gene (GRN, granulin prec...

2015
Tom C. van der Zanden

Graph constraint logic is a framework introduced by Hearn and Demaine [6], which provides several problems that are often a convenient starting point for reductions. We study the parameterized complexity of Constraint Graph Satisfiability and both bounded and unbounded versions of Nondeterministic Constraint Logic (NCL) with respect to solution length, treewidth and maximum degree of the underl...

Journal: :Brain research 2004
Charlie C Pontikis Claire V Cella Nisha Parihar Ming J Lim Shubhodeep Chakrabarti Hannah M Mitchison William C Mobley Payam Rezaie David A Pearce Jonathan D Cooper

Mouse models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human disorder, with widespread regional atrophy and significant loss of GABAergic interneurons in the hippocampus and neocortex. Reactive gliosis is a characteristic of all forms of NCL, but it is unclear whether glial activation precedes or is triggered by neuronal loss. To explore this issue we undertook detail...

Journal: :Nature communications 2013
Lena Veit Andreas Nieder

Despite the lack of a layered neocortex and fundamental differences in endbrain organization in birds compared with mammals, intelligent species evolved from both vertebrate classes. Among birds, corvids show exceptional cognitive flexibility. Here we explore the neuronal foundation of corvid cognition by recording single-unit activity from an association area known as the nidopallium caudolate...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2015
Lena Veit Galyna Pidpruzhnykova Andreas Nieder

The ability to form associations between behaviorally relevant sensory stimuli is fundamental for goal-directed behaviors. We investigated neuronal activity in the telencephalic area nidopallium caudolaterale (NCL) while two crows (Corvus corone) performed a delayed association task. Whereas some paired associates were familiar to the crows, novel associations had to be learned and mapped to th...

Journal: :Biochemical Society transactions 2010
Jonathan D Cooper

The NCLs (neuronal ceroid lipofuscinoses) (also known as Batten disease) are a group of at least ten fatal inherited storage disorders. Despite the identification of many of the disease-causing genes, very little is known about the underlying disease mechanisms. However, now that we have mouse or large-animal models for most forms of NCL, we can investigate pathogenesis and compare what happens...

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