Lysosomes as coordinators of cellular catabolism, metabolic signalling and organ physiology
C Settembre, RM Perera - Nature Reviews Molecular Cell Biology, 2024 - nature.com
Every cell must satisfy basic requirements for nutrient sensing, utilization and recycling
through macromolecular breakdown to coordinate programmes for growth, repair and stress …
through macromolecular breakdown to coordinate programmes for growth, repair and stress …
Mutant mice with rod-specific VPS35 deletion exhibit retinal α-synuclein pathology-associated degeneration
C Fu, N Yang, JZ Chuang, N Nakajima, S Iraha… - Nature …, 2024 - nature.com
Abstract Vacuolar protein sorting 35 (VPS35), the core component of the retromer complex
which regulates endosomal trafficking, is genetically linked with Parkinson's disease (PD) …
which regulates endosomal trafficking, is genetically linked with Parkinson's disease (PD) …
A genetically-encoded fluorescence-based reporter to spatiotemporally investigate mannose-6-phosphate pathway
M Bhat, A Nambiar, L Edakkandiyil… - Molecular Biology of …, 2024 - Am Soc Cell Biol
Maintenance of a pool of active lysosomes with acidic pH and degradative hydrolases is
crucial for cell health. Abnormalities in lysosomal function are closely linked to diseases …
crucial for cell health. Abnormalities in lysosomal function are closely linked to diseases …
TRPML1 activation ameliorates lysosomal phenotypes in CLN3 deficient retinal pigment epithelial cells
D Wünkhaus, R Tang, K Nyame, NN Laqtom… - Scientific Reports, 2024 - nature.com
Mutations in the lysosomal membrane protein CLN3 cause Juvenile Neuronal Ceroid
Lipofuscinosis (JNCL). Activation of the lysosomal ion channel TRPML1 has previously …
Lipofuscinosis (JNCL). Activation of the lysosomal ion channel TRPML1 has previously …
CLN3 deficiency leads to neurological and metabolic perturbations during early development
U Heins-Marroquin, RR Singh… - Life Science …, 2024 - life-science-alliance.org
Juvenile neuronal ceroid lipofuscinosis (or Batten disease) is an autosomal recessive, rare
neurodegenerative disorder that affects mainly children above the age of 5 yr and is most …
neurodegenerative disorder that affects mainly children above the age of 5 yr and is most …
Downregulation of Protease Cathepsin D and Upregulation of Pathologic α-Synuclein Mediate Paucity of DNAJC6-Induced Degeneration of Dopaminergic Neurons
CC Chiu, YL Chen, YH Weng, SY Liu, HL Li… - International Journal of …, 2024 - mdpi.com
A homozygous mutation of the DNAJC6 gene causes autosomal recessive familial type 19
of Parkinson's disease (PARK19). To test the hypothesis that PARK19 DNAJC6 mutations …
of Parkinson's disease (PARK19). To test the hypothesis that PARK19 DNAJC6 mutations …
Loss of the lysosomal protein CLN3 modifies the lipid content of the nuclear envelope leading to DNA damage and activation of YAP1 pro-apoptotic signaling
N Domingues, J Pires, SR Freire, NJ Herz, T Huynh… - bioRxiv, 2024 - ncbi.nlm.nih.gov
Batten disease is characterized by early-onset blindness, juvenile dementia and death
during the second decade of life. The most common genetic causes are mutations in the …
during the second decade of life. The most common genetic causes are mutations in the …
New tools can propel research in lysosomal storage diseases
E Hertz, LM Glasstetter, Y Chen, E Sidransky - Molecular Genetics and …, 2023 - Elsevier
Historically, the clinical manifestations of lysosomal storage diseases offered an early
glimpse into the essential digestive functions of the lysosome. However, it was only recently …
glimpse into the essential digestive functions of the lysosome. However, it was only recently …
[HTML][HTML] CD8α-CI-M6PR Particle Motility Assay to Study the Retrograde Motion of CI-M6PR Receptors in Cultured Living Cells
S Rawat, M Sharma - Bio-protocol, 2024 - ncbi.nlm.nih.gov
The cation-independent mannose 6-phosphate receptors (CI-M6PR) bind newly
synthesized mannose 6-phosphate (Man-6-P)-tagged enzymes in the Golgi and transport …
synthesized mannose 6-phosphate (Man-6-P)-tagged enzymes in the Golgi and transport …