BACKGROUND G-quadruplexes (G4s) are four-stranded nucleic acid structures that form in G-rich sequences. Nucleolin (NCL) is a cellular protein reported for its functions upon G4 recognition, such as induction of neurodegenerative diseases, tumor and virus mechanisms activation. We here aimed at defining NCL/G4 binding determinants. METHODS Electrophoresis mobility shift assay was used to dete...

The proteins ClC-6 and ClC-7 are expressed in the endosomal-lysosomal system. Because Clcn6-deficient mice display some features of neuronal ceroid lipofuscinosis (NCL), CLCN6 may be a candidate gene for novel forms of NCL. Using landmarks of disease progression from NCL mouse models as a guide, we examined neuropathologic alterations in the central nervous system of Clcn6(-/-), Clcn7(-/-), and...

The execution of action sequences is the basis of most behavior. However, little is known about the neural foundation of visuomotor sequence execution in birds, although pigeons are a classic model animal to study sequence learning and production. Recently, we identified two structures in the pigeon brain, the nidopallium intermedium medialis pars laterale (NIML) and the nidopallium caudolatera...

Abstract Anti–correlation has been used in training neural network ensembles. Negative correlation learning (NCL) is the state of the art anti–correlation measure. An alternative anti–correlation measure, RTQRT–NCL, which shows significant improvements on our test examples for both artificial neural networks (ANN) and genetic programming (GP) learning machines, is presented. We analyze the beha...

44 The execution of action sequences is the basis of most behavior. However, little is 45 known about the neural foundation of visuomotor sequence execution in birds, 46 although pigeons are a classic model animal to study sequence learning and 47 production. Recently, we identified two structures in the pigeon brain, the nidopallium 48 intermedium medialis pars laterale (NIML) and the nidopall...

PURPOSE Late juvenile neuronal ceroid lipofuscinosis (NCL) is a lysosomal neurodegenerative disorder caused by the accumulation of lipopigment in neurons. Our purpose was to characterize the MR imaging and spectroscopic findings in three children with late infantile NCL. METHODS Three children with late infantile NCL and three age-matched control subjects were examined by MR imaging and by lo...

The neuronal ceroid lipofuscinoses (NCLs) are devastating inherited progressive neurodegenerative diseases, with most forms having a childhood onset of clinical signs. The NCLs are characterized by progressive cognitive and motor decline, vision loss, seizures, respiratory and swallowing impairment, and ultimately premature death. Different forms of NCL result from mutations in at least 13 gene...

Songbirds possess acute vision. How higher brain centres represent basic and parameterised visual stimuli to process sensory signals according to their behavioural importance has not been studied in a systematic way. We therefore examined how carrion crows (Corvus corone) and their nidopallial visual neurons process global visual motion information in dynamic random-dot displays during a delaye...

We report a 17-year-old female with juvenile neuronal ceroid-lipofuscinosis (NCL) accompanied by hypertrophic cardiomyopathy (HCM) and left ventricular noncompaction (LVNC). Within our knowledge, this is the first reported case of juvenile NCL with LVNC, and the youngest case of HCM diagnosed by ultrasound. Juvenile NCL is a progressive hereditary disease involving multi-organ accumulation of c...

Anti–correlation has been used in training neural network ensembles. Negative correlation learning (NCL) is the state of the art anti–correlation measure. We propose an alternative anti–correlation measure, RTQRT–NCL, which shows signi£cant improvements on our test example, particularly with larger ensembles. We analyze the behavior of the negative correlation measure and derive a theoretical e...