Matches in SemOpenAlex for { <https://semopenalex.org/work/W1980162202> ?p ?o ?g. }
- W1980162202 endingPage "1138" @default.
- W1980162202 startingPage "1124" @default.
- W1980162202 abstract "Intracellular pathways leading to neuronal degeneration are poorly understood in the juvenile neuronal ceroid lipofuscinosis (JNCL, Batten disease), caused by mutations in the CLN3 gene. To elucidate the early pathology, we carried out comparative global transcript profiling of the embryonic, primary cultures of the Cln3-/- mouse neurons. Statistical and functional analyses delineated three major cellular pathways or compartments affected: mitochondrial glucose metabolism, cytoskeleton, and synaptosome. Further functional studies showed a slight mitochondrial dysfunction and abnormalities in the microtubule cytoskeleton plus-end components. Synaptic dysfunction was also indicated by the pathway analysis, and by the gross upregulation of the G protein beta 1 subunit, known to regulate synaptic transmission via the voltage-gated calcium channels. Intracellular calcium imaging showed a delay in the recovery from depolarization in the Cln3-/- neurons, when the N-type Ca2+ channels had been blocked. The data suggests a link between the mitochondrial dysfunction and cytoskeleton-mediated presynaptic inhibition, thus providing a foundation for further investigation of the disease mechanism underlying JNCL disease." @default.
- W1980162202 created "2016-06-24" @default.
- W1980162202 creator A5007296827 @default.
- W1980162202 creator A5009398385 @default.
- W1980162202 creator A5013522956 @default.
- W1980162202 creator A5016001137 @default.
- W1980162202 creator A5054298399 @default.
- W1980162202 creator A5056107874 @default.
- W1980162202 creator A5068062411 @default.
- W1980162202 creator A5069782141 @default.
- W1980162202 date "2006-08-28" @default.
- W1980162202 modified "2023-10-14" @default.
- W1980162202 title "Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments" @default.
- W1980162202 cites W1503369988 @default.
- W1980162202 cites W1538879480 @default.
- W1980162202 cites W1550967330 @default.
- W1980162202 cites W1554617917 @default.
- W1980162202 cites W1956706703 @default.
- W1980162202 cites W1974359647 @default.
- W1980162202 cites W1978680741 @default.
- W1980162202 cites W1979216037 @default.
- W1980162202 cites W1981585359 @default.
- W1980162202 cites W1981845284 @default.
- W1980162202 cites W1984979116 @default.
- W1980162202 cites W1995390203 @default.
- W1980162202 cites W1997619492 @default.
- W1980162202 cites W2009451871 @default.
- W1980162202 cites W2015584925 @default.
- W1980162202 cites W2017810117 @default.
- W1980162202 cites W2023512898 @default.
- W1980162202 cites W2026776024 @default.
- W1980162202 cites W2027417884 @default.
- W1980162202 cites W2031775911 @default.
- W1980162202 cites W2046372907 @default.
- W1980162202 cites W2047685288 @default.
- W1980162202 cites W2053476833 @default.
- W1980162202 cites W2055022577 @default.
- W1980162202 cites W2055893119 @default.
- W1980162202 cites W2072073919 @default.
- W1980162202 cites W2074696689 @default.
- W1980162202 cites W2088234686 @default.
- W1980162202 cites W2091735806 @default.
- W1980162202 cites W2100196074 @default.
- W1980162202 cites W2103017472 @default.
- W1980162202 cites W2107277218 @default.
- W1980162202 cites W2107846158 @default.
- W1980162202 cites W2108155784 @default.
- W1980162202 cites W2110241818 @default.
- W1980162202 cites W2111396800 @default.
- W1980162202 cites W2118986914 @default.
- W1980162202 cites W2124772147 @default.
- W1980162202 cites W2127868482 @default.
- W1980162202 cites W2138247507 @default.
- W1980162202 cites W2139861422 @default.
- W1980162202 cites W2140075873 @default.
- W1980162202 cites W2140834763 @default.
- W1980162202 cites W2141010238 @default.
- W1980162202 cites W2142003218 @default.
- W1980162202 cites W2144151547 @default.
- W1980162202 cites W2145213346 @default.
- W1980162202 cites W2156879185 @default.
- W1980162202 cites W2160879813 @default.
- W1980162202 cites W2165298990 @default.
- W1980162202 cites W2166921996 @default.
- W1980162202 cites W2168808956 @default.
- W1980162202 cites W2170681065 @default.
- W1980162202 cites W32417562 @default.
- W1980162202 cites W4246573955 @default.
- W1980162202 cites W4322702932 @default.
- W1980162202 doi "https://doi.org/10.1002/jnr.21015" @default.
- W1980162202 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16941499" @default.
- W1980162202 hasPublicationYear "2006" @default.
- W1980162202 type Work @default.
- W1980162202 sameAs 1980162202 @default.
- W1980162202 citedByCount "71" @default.
- W1980162202 countsByYear W19801622022012 @default.
- W1980162202 countsByYear W19801622022013 @default.
- W1980162202 countsByYear W19801622022014 @default.
- W1980162202 countsByYear W19801622022015 @default.
- W1980162202 countsByYear W19801622022016 @default.
- W1980162202 countsByYear W19801622022017 @default.
- W1980162202 countsByYear W19801622022018 @default.
- W1980162202 countsByYear W19801622022019 @default.
- W1980162202 countsByYear W19801622022020 @default.
- W1980162202 countsByYear W19801622022021 @default.
- W1980162202 countsByYear W19801622022022 @default.
- W1980162202 crossrefType "journal-article" @default.
- W1980162202 hasAuthorship W1980162202A5007296827 @default.
- W1980162202 hasAuthorship W1980162202A5009398385 @default.
- W1980162202 hasAuthorship W1980162202A5013522956 @default.
- W1980162202 hasAuthorship W1980162202A5016001137 @default.
- W1980162202 hasAuthorship W1980162202A5054298399 @default.
- W1980162202 hasAuthorship W1980162202A5056107874 @default.
- W1980162202 hasAuthorship W1980162202A5068062411 @default.
- W1980162202 hasAuthorship W1980162202A5069782141 @default.
- W1980162202 hasConcept C104317684 @default.
- W1980162202 hasConcept C142669718 @default.
- W1980162202 hasConcept C142724271 @default.