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MetabolomicsLongitudinal Deep Multi-Omics Profiling in aCLN3Δex7/8Minipig Model Reveals Novel Biomarker Signatures for Batten Disease
Development of therapies for CLN3 Batten disease, a rare pediatric lysosomal storage disorder, has been hindered by the lack of etiological insights and translatable biomarkers to clinics. Here, we used a deep multi-omics approach to discover new biomarkers using longitudinal serum samples from a po...
2025-08-13 | MTBLS1107 | MetaboLights
ProteomicsModeling CLN3 Batten disease in astrocytes reveals dysfunctions in mitochondria, fatty acid metabolism and chromatin dynamics
CLN3 Batten disease is a severe pediatric neurodegenerative disorder caused by mutations in the CLN3 gene, most commonly a 1 kb deletion affecting exons 7 and 8. While research has focused on neuronal dysfunction, glial cells are increasingly recognized as key contributors to disease pathology. Amon...
ORGANISM(S): Homo sapiens (Human) 
2026-06-08 | PXD064202 | Pride
Batten disease 
UnknownNeurobehavioral features and natural history of juvenile neuronal ceroid lipofuscinosis (Batten disease).
Not available
S-EPMC3976549 | biostudies-literature
UnknownSpeech, Language and Non-verbal Communication in CLN2 and CLN3 Batten Disease.
Not available
S-EPMC11739554 | biostudies-literature
UnknownSearching for novel biomarkers using a mouse model of CLN3-Batten disease.
Not available
S-EPMC6080763 | biostudies-literature
ProteomicsLOSS OF THE BATTEN DISEASE PROTEIN CLN3 LEADS TO MIS-TRAFFICKING OF M6PR AND DEFECTIVE AUTOPHAGIC-LYSOSOMAL REFORMATION
Batten disease, one of the most devastating types of neurodegenerative lysosomal storage disorders, is caused by mutations in CLN3. Here, we show that CLN3 is a vesicular trafficking hub connecting the Golgi and lysosome compartments. Proteomic analysis reveals that CLN3 interacts with several endo-...
ORGANISM(S): Homo sapiens (Human) 
2023-06-20 | PXD031582 | Pride
Human Cln3 Batten Lysosome 
UnknownTherapeutic antisense oligonucleotide mitigates retinal dysfunction in a pig model of CLN3 Batten disease.
Not available
S-EPMC12585909 | biostudies-literature
UnknownNeurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease).
Not available
S-EPMC4915684 | biostudies-literature
UnknownTherapeutic efficacy of antisense oligonucleotides in mouse models of CLN3 Batten disease.
Not available
S-EPMC8008709 | biostudies-literature
UnknownRecognizing differentiating clinical signs of CLN3 disease (Batten disease) at presentation.
Not available
S-EPMC8359263 | biostudies-literature
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