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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 19 Aug 2016.

Ceroid-lipofuscinosis, neuronal 3

This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CLN2, CAN, nucleolin, HAD, ACID
Papers on CLN3
Sialoadhesin promotes neuroinflammation-related disease progression in two mouse models of CLN disease.
Martini et al., Würzburg, Germany. In Glia, Feb 2016
We now investigated the role of the inflammation-related cell adhesion molecule sialoadhesin (Sn) in Ppt1(-/-) and Cln3(-/-) mice, a model of the most frequent form, CLN3 disease.
Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease).
Wang et al., Lexington, United States. In Ann N Y Acad Sci, Feb 2016
Although ceroid-lipofuscinosis, neuronal 3 (CLN3) has been identified as the sole disease gene, the biochemical and cellular bases of JNCL and the functions of CLN3 are yet to be fully understood.
Fibrates inhibit the apoptosis of Batten disease lymphoblast cells via autophagy recovery and regulation of mitochondrial membrane potential.
Kim et al., Asan, South Korea. In In Vitro Cell Dev Biol Anim, Jan 2016
UNASSIGNED: Batten disease (BD; also known as juvenile neuronal ceroid lipofuscinosis) is a genetic disorder inherited as an autosomal recessive trait and is characterized by blindness, seizures, cognitive decline, and early death resulting from the inherited mutation of the CLN3 gene.
Genetics of the neuronal ceroid lipofuscinoses (Batten disease).
Cotman et al., London, United Kingdom. In Biochim Biophys Acta, Oct 2015
These genes encode lysosomal enzymes (CLN1, CLN2, CLN10, CLN13), a soluble lysosomal protein (CLN5), a protein in the secretory pathway (CLN11), two cytoplasmic proteins that also peripherally associate with membranes (CLN4, CLN14), and many transmembrane proteins with different subcellular locations (CLN3, CLN6, CLN7, CLN8, CLN12).
The neuronal ceroid lipofuscinoses program: A translational research experience in Argentina.
Noher de Halac et al., Córdoba, Argentina. In Biochim Biophys Acta, Oct 2015
Phenotypic studies comprised epileptic seizures and movement disorders, ophthalmology, neurophysiology, image analysis, rating scales, enzyme testing, and electron microscopy, carried out under a consensus algorithm; 2) DNA screening and validation of mutations in genes PPT1 (CLN1), TPP1 (CLN2), CLN3, CLN5, CLN6, MFSD8 (CLN7), and CLN8: characterization of variant types, novel/known mutations and polymorphisms; 3) Progress of the epidemiological picture in Latin America; and 4) NCL-like pathology studies in progress.
Cell biology of the NCL proteins: What they do and don't do.
Pearce et al., Sioux Falls, United States. In Biochim Biophys Acta, Oct 2015
NCL-associated proteins have been localized mostly in lysosomes (CLN1, CLN2, CLN3, CLN5, CLN7, CLN10, CLN12 and CLN13) but also in the Endoplasmic Reticulum (CLN6 and CLN8), or in the cytosol associated to vesicular membranes (CLN4 and CLN14).
The Voltage-Gated Proton Channel: A Riddle, Wrapped in a Mystery, inside an Enigma.
DeCoursey, Chicago, United States. In Biochemistry, Jul 2015
The main properties of the voltage-gated proton channel (HV1) are described in this review, along with what is known about how the channel protein structure accomplishes its functions.
Aptamers Binding to c-Met Inhibiting Tumor Cell Migration.
Hock et al., Darmstadt, Germany. In Plos One, 2014
CLN64 and a previously described single-stranded DNA (ssDNA) aptamer CLN3 exhibited high specificities and affinities to recombinant and cellular expressed c-Met.
Mepolizumab versus placebo for asthma.
Walters et al., Cardiff, United Kingdom. In Cochrane Database Syst Rev, 2014
There are no recommendations for the use of mepolizumab in adults or children in the recent update of the BTS/SIGN guidelines (BTS/SIGN 2014).
TRIXcell+, a new long-term boar semen extender containing whey protein with higher preservation capacity and litter size.
Reesink et al., Enkhuizen, Netherlands. In Open Vet J, 2013
Boar sperm diluted in TRIXcell+ maintains commercially acceptable motility (>60%) for 10 days, while swine sperm diluted in the semen preservation medium Beltsville Thawing Solution (BTS) maintains commercially acceptable motility (>60%) for 3-5 days for most boars.
Lipopolysaccharide-sensitive H+ current in dendritic cells.
Shumilina et al., Tübingen, Germany. In Am J Physiol Cell Physiol, 2012
Functional expression of voltage-gated proton (Hv1) channels is characterized, for the first time, in mouse bone marrow-derived dendritic cells.
Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartments.
Jalanko et al., Helsinki, Finland. In Cell Mol Life Sci, 2012
The data presented in this study provide novel insights into the role of CLN3 in late endosomal/lysosomal membrane transport.
Clinicopathological and biological significance of human voltage-gated proton channel Hv1 protein overexpression in breast cancer.
Li et al., Tianjin, China. In J Biol Chem, 2012
inhibition of Hv1 function via knockdown of Hv1 expression can effectively retard cancer growth
The voltage-gated proton channel Hv1 enhances brain damage from ischemic stroke.
Clapham et al., Boston, United States. In Nat Neurosci, 2012
Hv1-dependent reactive oxygen species production is responsible for a substantial fraction of brain damage at early time points after ischemic stroke.
Antagonistic gene transcripts regulate adaptation to new growth environments.
Hasty et al., San Diego, United States. In Proc Natl Acad Sci U S A, 2012
The spatial regulation of GAL1 and CLN3 transcripts is what allows growth to be maintained during fluctuations of glucose availability.
The effects of molecular noise and size control on variability in the budding yeast cell cycle.
Cross et al., New York City, United States. In Nature, 2007
The first step, which depends on the G1 cyclin gene CLN3, corresponds to noisy size control that extends G1 in small daughters, but is of negligible duration in mothers.
A small-molecule inhibitor of skeletal muscle myosin II.
Straight et al., Boston, United States. In Nat Cell Biol, 2002
The best inhibitor, N-benzyl-p-toluene sulphonamide (BTS), an aryl sulphonamide, inhibited the Ca2+-stimulated S1 ATPase, and reversibly blocked gliding motility.
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
Sherman et al., Rochester, United States. In Nat Genet, 1999
The CLN3 gene, which is responsible for Batten disease, has been positionally cloned.
Linkage of replication to start by the Cdk inhibitor Sic1.
Futcher et al., United States. In Science, 1996
Inactivation of Sic1 was the only nonredundant essential function of Clns, because a sic1 deletion rescued the inviability of the cln1 cln2 cln3 triple mutant.
Cell cycle control by a complex of the cyclin HCS26 (PCL1) and the kinase PHO85.
Morgan et al., San Francisco, United States. In Science, 1994
Passage through the G1 phase of the cell cycle is regulated by complexes of CDC28 and G1 cyclins (CLN1, CLN2, and CLN3).
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