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UnknownDisruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.
Not available
S-EPMC3335005 | biostudies-literature
UnknownThe gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein.
Not available
S-EPMC384927 | biostudies-literature
UnknownSustained Neural Stem Cell-Based Intraocular Delivery of CNTF Attenuates Photoreceptor Loss in the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis.
Not available
S-EPMC4439090 | biostudies-literature
UnknownDistinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cells.
Not available
S-EPMC3040763 | biostudies-literature
TranscriptomicsDistinct Early Molecular Responses to Mutations Causing vLINCL and JNCL Presage ATP Synthase Subunit c Accumulation in Cerebellar Cells
Variant late-infantile (vLINCL) and juvenile neuronal ceroid lipofuscinosis (JNCL) share clinical and pathological features, including lysosomal accumulation of mitochondrial ATP synthase subunit c, but the unrelated CLN6 and CLN3 genes may initiate disease via similar or distinct cellular processes...
ORGANISM(S): Mus musculus 
2011-02-01 | GSE24368 | GEO
TranscriptomicsDistinct Early Molecular Responses to Mutations Causing vLINCL and JNCL Presage ATP Synthase Subunit c Accumulation in Cerebellar Cells
Variant late-infantile (vLINCL) and juvenile neuronal ceroid lipofuscinosis (JNCL) share clinical and pathological features, including lysosomal accumulation of mitochondrial ATP synthase subunit c, but the unrelated CLN6 and CLN3 genes may initiate disease via similar or distinct cellular processes...
ORGANISM(S): Mus musculus 
2011-02-01 | E-GEOD-24368 | biostudies-arrayexpress
UnknownIntracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice.
Not available
S-EPMC7887332 | biostudies-literature
UnknownA murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.
Not available
S-EPMC3815212 | biostudies-literature
UnknownAn integrated wet-spinning system for continuous fabrication of high-strength nanocellulose long filaments.
Not available
S-EPMC10423198 | biostudies-literature
UnknownMutations in a novel CLN6-encoded transmembrane protein cause variant neuronal ceroid lipofuscinosis in man and mouse.
Not available
S-EPMC384912 | biostudies-literature
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