Ppt1‐deficiency dysregulates lysosomal Ca++ homeostasis contributing to pathogenesis in a mouse model of CLN1 disease
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Inherited Metabolic Disease"
DOI: 10.1002/jimd.12485
Abstract: Inactivating mutations in the PPT1 gene encoding palmitoyl‐protein thioesterase‐1 (PPT1) underlie the CLN1 disease, a devastating neurodegenerative lysosomal storage disorder. The mechanism of pathogenesis underlying CLN1 disease has remained elusive. PPT1 is a lysosomal enzyme,… read more here.
Keywords: lysosomal homeostasis; disease; cln1 disease; ppt1 deficiency ... See more keywords
Coordination of mitochondrial and lysosomal homeostasis mitigates inflammation and muscle atrophy during aging
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DOI: 10.1111/acel.13583
Abstract: Sarcopenia is one of the main factors contributing to the disability of aged people. Among the possible molecular determinants of sarcopenia, increasing evidences suggest that chronic inflammation contributes to its development. However, a key unresolved… read more here.
Keywords: inflammation; lysosomal homeostasis; muscle; atrophy ... See more keywords
Activation of Transcription Factor EB Alleviates Tubular Epithelial Cell Injury via Restoring Lysosomal Homeostasis in Diabetic Nephropathy
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DOI: 10.1155/2022/2812493
Abstract: Disruption of lysosomal homeostasis contributes to the tubulopathy of diabetic nephropathy; however, its underlying mechanisms remain unclear. Herein, we report that decreased activity of transcription factor EB (TFEB) is responsible for the disturbed lysosome biogenesis… read more here.
Keywords: lysosomal homeostasis; transcription factor; tubular epithelial; diabetic nephropathy ... See more keywords
Safeguarding Lysosomal Homeostasis by DNAJC5/CSPα-Mediated Unconventional Protein Secretion and Endosomal Microautophagy
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Cell and Developmental Biology"
DOI: 10.3389/fcell.2022.906453
Abstract: Neuronal ceroid lipofuscinosis (NCL) is a collection of genetically inherited neurological disorders characterized by vision loss, seizure, brain death, and premature lethality. At the cellular level, a key pathologic hallmark of NCL is the build-up… read more here.
Keywords: lysosomal homeostasis; dnajc5 csp; endosomal microautophagy; unconventional protein ... See more keywords
TFEB Probably Involved in Midazolam-Disturbed Lysosomal Homeostasis and Its Induced β-Amyloid Accumulation
Sign Up to like & getrecommendations! Published in 2019 at "Frontiers in Human Neuroscience"
DOI: 10.3389/fnhum.2019.00108
Abstract: Alzheimer’s disease (AD) is one of the most common neurodegenerative diseases, and β-amyloid (Aβ) plays a leading role in the pathogenesis of AD. The transcription factor EB (TFEB), a main regulating factor of autophagy and… read more here.
Keywords: midazolam disturbed; disturbed lysosomal; lysosomal homeostasis; involved midazolam ... See more keywords