Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)
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We report here the intracellular (pHi) and lysosomal pH in fibroblasts of six forms of neuronal ceroid lipofuscinoses (NCLs). Acid extrusion rate and pH(i) values were measured by the membrane-permeant acetoxymethyl ester of the indicator dye, 2',7'-bis(carboxyethyl)-5-(and-6)-carboxy-fluorescein (BCECF) and lysosomal pH by a spectrofluorometric assay utilizing a novel acidotropic probe, Lysosensor yellow/blue. Intracellular pH was normal in all NCLs. Elevated lysosomal pH was detected in all NCL forms except CLN2 and CLN8. Elevated pH most probably disturbs the catalytic activity of lysosomes and is one important factor in explaining accumulation of ceroid and lipofuscin-like autofluorescent lipopigments characteristic of NCLs. Using the novel spectrofluorometric assay introduced in this study provides a fast and repeatable technique to measure intralysosomal pH from cell suspensions.
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Overall, the odds ratio for developing PD associated with GBA (OR 5.4) is much higher than the other genes described above, with the genes encoding proteins further away from GBA in sphingolipid processing having weaker and weaker evidence.
“Overall, the odds ratio for developing PD associated with GBA (OR 5.4) is much higher than the other genes described above, with the genes encoding proteins further away from GBA in sphingolipid processing having weaker and weaker evidence (Figure 4, right panel).72,108”
From The lysosome and proteostatic stress at the intersection of pediatric neurological disorders and adult neurodegenerative diseases (2025) · cites this paper as [72]
Heterozygosity for GBA mutations increases risk for Parkinson's Disease and is associated with a younger age of onset.
“In 2004, a landmark study found that heterozygosity for GBA mutations increased risk for Parkinson’s Disease and was associated with a younger age of onset.72”
From The lysosome and proteostatic stress at the intersection of pediatric neurological disorders and adult neurodegenerative diseases (2025) · cites this paper as [72]
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References 42 · Cited by 211
- Authors
- Holopainen, J. M., Saarikoski, J., Kinnunen, P. K. J., & Järvelä, I.
- Affiliations
- University of Helsinki (FI)
- Helsinki University Hospital (FI)
- Venue
- European Journal of Biochemistry
- Publisher
- Wiley
- Published
- 2001-11-15
- Volume
- 268
- Issue
- 22
- Pages
- 5851-5856
- ISSN
- 0014-2956 (ISSN-L)
- 1432-1033
- OpenAlex
- W1597721823
- Cited by
- 211
- References
- 42
- Citations by year
- 2026: 7 · 2025: 6 · 2024: 4 · 2023: 5 · 2022: 7 · 2021: 11 · 2020: 11 · 2019: 10 · 2018: 10 · 2017: 7 · 2016: 7 · 2015: 8 · 2014: 5 · 2013: 10 · 2012: 8
- FWCI
- 3.39
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- European Journal of Biochemistry (publishedVersion)
- PubMed (publishedVersion)
- STM:n Hallinnonalan avoin julkaisuarkisto (Julkari) (submittedVersion)
- Topics
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Pathology and Forensic Medicine › Biomedical Research and Pathophysiology
- Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
- Keywords
- neuronal ceroid lipofuscinosis, lysosomal pH, intracellular pH, fibroblasts, lysosomal acidification, BCECF, lysosomal enzyme activity, lipofuscin accumulation, CLN2, CLN8, lysosomal storage disease
- MeSH terms
- Tripeptidyl-Peptidase 1, Cell Line, Coloring Agents, Fluoresceins, Humans, Hydrogen-Ion Concentration, Lysosomes, Neuronal Ceroid-Lipofuscinoses, Oxazoles
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