نتایج جستجو برای: ncl
تعداد نتایج: 963 فیلتر نتایج به سال:
Mutations in the CLN6 gene cause a variant late infantile form of neuronal ceroid lipofuscinosis (NCL; Batten disease). CLN6 loss leads to disease clinically characterized by vision impairment, motor and cognitive dysfunction, and seizures. Accumulating evidence suggests that alterations in metal homeostasis and cellular signaling pathways are implicated in several neurodegenerative and develop...
NCL2, the neuronal ceroid lipofuscinosis (NCL) with a classical late infantile onset (LINCL or JanskyBielschowsky disease, MIM 204500) is among the most common childhood neurodegenerative disorders. Usually, affected children show generalised tonic-clonic and/or myoclonic seizures starting by 2 to 3 years of age as the initial, and the most noticeable, clinical symptom. This is followed by regr...
195pt and 1h nmr has been used to show that addition of chlorine and bromine to [pt(cn)4]2_ in presence of perchloric acid in water results in cyanide and water scrambling with formation of eleven complexes of the type [pt(cn)4-ncl(h2o)n+1](n-1)+ (n=0, 1, 2, 3, 4). addition of nbu4oh to this solution gives (nbu4)2[pt(cn)4cl(oh)]. nmr measurements in acetone also show cyanide and hydroxyl group ...
In their review in PNAS, Allen and Fortin use a comparative neuroanatomical approach to gain insight into the evolution of episodic memory (1). The authors suggest that, as in humans, episodic-like memory in nonhuman mammals depends on a system consisting of the hippocampus, parahippocampal region, and prefrontal cortex (PFC), and, in birds, a similar system consisting of the hippocampus, area ...
Acute myeloid leukemia (AML) is a heterogeneous disease. Prognosis is mainly influenced by patient age at diagnosis and cytogenetic alterations, two of the main factors currently used in AML patient risk stratification. However, additional criteria are required to improve the current risk classification and better adapt patient care. In neoplastic cells, ribosome biogenesis is increased to sust...
The neostriatum caudolaterale (NCL) of birds is thought to be equivalent to the mammalian prefrontal cortex (PFC) due to its dense dopaminergic innervation, its associative structure, and its importance for cognitive tasks which are known to be affected in mammals with prefrontal lesions. The aim of the present study was to analyze the functional importance of the NCL and its main thalamic affe...
Neuronal ceroid lipofuscinosis (NCL) is an inherited, neurodegenerative lysosomal disease that causes premature death. The present study describes the clinical and molecular epidemiologic findings of NCL in Border Collies in Japan for 12 years, between 2000 and 2011. The number of affected dogs was surveyed, and their clinical characteristics were analyzed. In 4 kennels with affected dogs, the ...
Ribosome biogenesis takes place in the nucleolus, the size of which is often coordinated with cell growth and development. However, how metazoans control nucleolar size remains largely unknown. Caenorhabditis elegans provides a good model to address this question owing to distinct tissue distribution of nucleolar sizes and a mutant, ncl-1, which exhibits larger nucleoli than wild-type worms. He...
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