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Article

Defective lysosomal arginine transport in juvenile Batten disease.

Human molecular genetics - 1 Dec 2005

Ramirez-Montealegre Denia, Pearce David A

Abstract excerpt

Mutations in the CLN3 gene, which encodes a lysosomal membrane protein, are responsible for the neurodegenerative disorder juvenile Batten disease. A previous study on the yeast homolog to CLN3, designated Btn1p, revealed a potential role for CLN3 in the transport of arginine into the yeast vacuole, the equivalent organelle to the mammalian lysosome. Lysosomes isolated from lymphoblast cell lines, established...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 16251196
DOI: 10.1093/hmg/ddi406

Genes

CLN3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Defective lysosomal arginine transport in juvenile Batten disease.PMID 16251196Read ↗

Absence of Btn1p in the yeast model for juvenile Batten disease may cause arginine to become toxic to yeast cells.Semantic proximity Read ↗

Defective intracellular transport of CLN3 is the molecular basis of Batten disease (JNCL)Semantic proximity Read ↗

Biosynthesis and Intracellular Targeting of the CLN3 Protein Defective in Batten DiseaseSemantic proximity Read ↗

Mutated genes in juvenile and variant late infantile neuronal ceroid lipofuscinoses encode lysosomal proteins.Semantic proximity Read ↗

Nitric oxide signaling is disrupted in the yeast model for Batten disease.Semantic proximity Read ↗

Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosisSemantic proximity Read ↗

Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].Semantic proximity Read ↗

Batten disease: evaluation of CLN3 mutations on protein localization and functionSemantic proximity Read ↗

A mouse gene knockout model for juvenile ceroid-lipofuscinosis (Batten disease).Semantic proximity Read ↗

Loss of the lysosomal protein CLN3 modifies the lipid content of the nuclear envelope leading to DNA damage and activation of YAP1 pro-apoptotic signalingSemantic proximity Read ↗

The Batten disease protein CLN3 is important for stress granules dynamics and translational activitySemantic proximity Read ↗

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