ReviewBrain pathology (Zurich, Switzerland)2004
The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.
Review in Brain pathology (Zurich, Switzerland), 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 42 citations in OpenAlex.
- Neuronal lipofuscinosis caused by Kufs disease/CLN4 DNAJC5 mutations but not by a CSPα/DNAJC5 deficiency.Science advances · 2025Article
- Article
- Oxidative stress status accompanying diabetic bladder cystopathy results in the activation of protein degradation pathways.BJU international · 2011Article
- Atypical CLN2 with later onset and prolonged course: a neuropathologic study showing different sensitivity of neuronal subpopulations to TPP1 deficiency.Acta neuropathologica · 2008Article
- Genetics and epilepsy.Dialogues in clinical neuroscience · 2008Review
- Proteomic analysis of cathepsin B- and L-deficient mouse brain lysosomes.Biochimica et biophysica acta · 2007Article
- Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.Proceedings of the National Academy of Sciences of the United States of America · 2006Article
- Batten disease: features to facilitate early diagnosis.The British journal of ophthalmology · 2006Article
- Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration.The EMBO journal · 2005Article
- Batten's Disease: A Seizure Disorder's Battle for Diagnosis.Annals of Indian Academy of NeurologyArticle
- Experimental priapism is associated with increased oxidative stress and activation of protein degradation pathways in corporal tissue.International journal of impotence researchArticle
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Neuronal ceroid lipofuscinoses are a group of diseases characterized by accumulation of hydrophobic proteins in lysosomes of neurons and other types of cells. NCLs are caused by at least 8 mutant genes (CLN1-CLN8), though CLN4 and CLN7 have not yet been identified. Except for Cln1p, the protein encoded by CLN1, the defective proteins are associated with lysosomal accumulation of mitochondrial ATP synthase subunit c. Cln1p and Cln2p are soluble lysosomal enzymes, targeted to lysosomes in a mannose 6-phosphate dependent manner. Mutations in the lysosomal protease cathepsin D cause another NCL. Cln3p, Cln5p, Cln6p and Cln8p are thought to be transmembrane proteins. Cln3p and Cln5p are localized in the endosome-lysosomal compartment. Deficiency of endosomal membrane protein CLC-3, a member of the chloride channel family, causes NCL-like phenotype and lysosomal storage of subunit c. Herein, we review the features of NCL and NCL-related proteins and discuss the involvement of the proteins in lysosomal degradation of subunit c.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.