Results 111 to 120 of about 1,071 (153)
Loss of the ER-cargo protein CLN8 increases severity of acute pancreatitis and upregulates ER-stress and ER-phagy. [PDF]
Mol BiomedZierke L +9 more
europepmc +1 more source
Discovery and biosynthesis of biffamycin A – a novel glycotetrapeptide antibiotic
Brigham MW +9 more
europepmc +1 more source
An altered secretome is an early marker of the pathogenesis of CLN6 Batten disease [PDF]
Journal of Neurochemistry, 2021 AbstractNeuronal ceroid lipofuscinoses (NCLs) are a group of inherited childhood neurodegenerative disorders. In addition to the accumulation of auto‐fluorescent storage material in lysosomes, NCLs are largely characterised by region‐specific neuroinflammation that can predict neuron loss.
Hannah Best +2 more
exaly +3 more sources
CLN6 deficiency causes selective changes in the lysosomal protein composition [PDF]
Proteomics, 2021 Abstract Neuronal ceroid lipofuscinoses (NCLs) collectively account for the highest prevalence of inherited neurodegenerative diseases in childhood. This disease group is classified by the deposition of similar autofluorescence storage material in lysosomes that is accompanied by seizures, blindness and premature ...
Marc Sylvester, Markus Damme
exaly +3 more sources
A CLN6-CLN8 complex recruits lysosomal enzymes at the ER for Golgi transfer [PDF]
Journal of Clinical Investigation, 2020 Lysosomal enzymes are synthesized in the endoplasmic reticulum (ER) and transferred to the Golgi complex by interaction with the Batten disease protein CLN8 (ceroid lipofuscinosis, neuronal, 8). Here we investigated the relationship of this pathway with CLN6, an ER-associated protein of unknown function that is defective in a different Batten disease ...
Marco Sardiello +2 more
exaly +5 more sources