Results 71 to 80 of about 17,019 (205)

Rapid Clathrin-Mediated Uptake of Recombinant α-Gal-A to Lysosome Activates Autophagy

Biomolecules, 2020
Enzyme replacement therapy (ERT) with recombinant alpha-galactosidase A (rh-α-Gal A) is the standard treatment for Fabry disease (FD). ERT has shown a significant impact on patients; however, there is still morbidity and mortality in FD, resulting in ...
Margarita M. Ivanova, Julia Dao, Neil Kasaci, Benjamin Adewale, Jacqueline Fikry, Ozlem Goker-Alpan   +5 more
doaj   +1 more source

Juvenile CLN3 disease is a lysosomal cholesterol storage disorder: similarities with Niemann-Pick type C diseaseResearch in context

EBioMedicine, 2023
Summary: Background: The most common form of neuronal ceroid lipofuscinosis (NCL) is juvenile CLN3 disease (JNCL), a currently incurable neurodegenerative disorder caused by mutations in the CLN3 gene.
Jacinda Chen, Rajesh Kumar Soni, Yimeng Xu, Sabrina Simoes, Feng-Xia Liang, Laura DeFreitas, Robert Hwang, Jr., Jorge Montesinos, Joseph H. Lee, Estela Area-Gomez, Renu Nandakumar, Badri Vardarajan, Catherine Marquer   +12 more
doaj   +1 more source

The Kangaroo Cation-independent Mannose 6-Phosphate Receptor Binds Insulin-like Growth Factor II with Low Affinity [PDF]

Journal of Biological Chemistry, 1999
The mammalian cation-independent mannose 6-phosphate receptor (CI-MPR) binds mannose 6-phosphate-bearing glycoproteins and insulin-like growth factor (IGF)-II. However, the CI-MPR from the opossum has been reported to bind bovine IGF-II with low affinity (Dahms, N. M., Brzycki-Wessell, M. A., Ramanujam, K. S., and Seetharam, B. (1993) Endocrinology 133,
Catherine Yandell, Andrew Dunbar, John F. Wheldrake, Zee Upton   +3 more
openalex   +4 more sources

Golgi-associated retrograde protein (GARP) complex-dependent endosomes to trans Golgi network retrograde trafficking is controlled by Rab4b

Cellular & Molecular Biology Letters
Background The trafficking of cargoes from endosomes to the trans-Golgi network requires numerous sequential and coordinated steps. Cargoes are sorted into endosomal-derived carriers that are transported, tethered, and fused to the trans-Golgi network ...
Jérôme Gilleron, Abderrahman Chafik, Sandra Lacas-Gervais, Jean-François Tanti, Mireille Cormont   +4 more
doaj   +1 more source

Maternal transmission of a humanised Igf2r allele results in an Igf2 dependent hypomorphic and non-viable growth phenotype.

PLoS ONE, 2013
The cation independent mannose 6-phosphate/insulin-like growth factor 2 receptor (IGF2R) functions in the transportation and regulation of insulin-like growth factor 2 (IGF2) and mannose 6-phosphate modified proteins.
Jennifer Hughes, Susana Frago, Claudia Bühnemann, Emma J Carter, A Bassim Hassan   +4 more
doaj   +1 more source

Reduced LRRK2 in association with retromer dysfunction in post-mortem brain tissue from LRRK2 mutation carriers [PDF]

, 2017
Missense mutations in leucine-rich repeat kinase 2 (LRRK2) are pathogenic for familial Parkinson's disease. However, it is unknown whether levels of LRRK2 protein in the brain are altered in patients with LRRK2-associated Parkinson's disease.
Dzamko, N, Halliday, GM, Hasegawa, K, Holton, JL, Jeremy Nichols, R, Mash, DC, Murayama, S, Paul, J, Perera, G, Takahashi-Fujigasaki, J, Uchino, A, Vonsattel, G, Zhao, Y   +12 more
core   +1 more source

CD8α-CI-M6PR Particle Motility Assay to Study the Retrograde Motion of CI-M6PR Receptors in Cultured Living Cells

Bio-Protocol
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 them to late endosomes/lysosomes, providing them with degradative functions.
Shalini Rawat, Mahak Sharma
doaj   +1 more source

BMN 250, a fusion of lysosomal alpha-N-acetylglucosaminidase with IGF2, exhibits different patterns of cellular uptake into critical cell types of Sanfilippo syndrome B disease pathogenesis.

PLoS ONE, 2019
Sanfilippo syndrome type B (Sanfilippo B; Mucopolysaccharidosis type IIIB) occurs due to genetic deficiency of lysosomal alpha-N-acetylglucosaminidase (NAGLU) and subsequent lysosomal accumulation of heparan sulfate (HS), which coincides with devastating
Gouri Yogalingam, Amanda R Luu, Heather Prill, Melanie J Lo, Bryan Yip, John Holtzinger, Terri Christianson, Mika Aoyagi-Scharber, Roger Lawrence, Brett E Crawford, Jonathan H LeBowitz   +10 more
doaj   +1 more source

Systematic mutational analysis of the cation-independent mannose 6-phosphate/insulin-like growth factor II receptor cytoplasmic domain - An acidic cluster containing a key aspartate is important for function in lysosomal enzyme sorting [PDF]

, 1997
We have used systematic mutational analysis to identify signals in the 166-residue murine cation-independent mannose 6-phosphate/insulin-like growth factor II receptor cytoplasmic domain required for efficient sorting of lysosomal enzymes.
Chen, Hong Jung, Lobel, Peter, Yuan, Jing   +2 more
core  

Quantitative analysis of insulin-like growth factor 2 receptor and insulin-like growth factor binding proteins to identify control mechanisms for insulin-like growth factor 1 receptor phosphorylation [PDF]

, 2016
Experimental results for the determination of internalization rates for IGF1-IGF1R, IGF2-IGF1R, IGF2-IGF2R.
Dan Tian, Isaiah Mitchell, Pamela K. Kreeger   +2 more
core   +5 more sources