Identification of a fourth mannose 6-phosphate binding site in the cation-independent mannose 6-phosphate receptor. [PDF]
Glycobiology, 2015 The 300 kDa cation-independent mannose 6-phosphate receptor (CI-MPR) plays an essential role in lysosome biogenesis by targeting ∼ 60 different phosphomannosyl-containing acid hydrolases to the lysosome.
Olson LJ +6 more
europepmc +6 more sources
Engineering of GlcNAc-1-Phosphotransferase for Production of Highly Phosphorylated Lysosomal Enzymes for Enzyme Replacement Therapy [PDF]
Molecular Therapy: Methods & Clinical Development, 2017 Several lysosomal enzymes currently used for enzyme replacement therapy in patients with lysosomal storage diseases contain very low levels of mannose 6-phosphate, limiting their uptake via mannose 6-phosphate receptors on the surface of the deficient ...
Lin Liu +3 more
doaj +4 more sources
A mutant of phosphomannomutase1 retains full enzymatic activity, but is not activated by IMP: Possible implications for the disease PMM2-CDG. [PDF]
PLoS ONE, 2017 The most frequent disorder of glycosylation, PMM2-CDG, is caused by a deficiency of phosphomannomutase activity. In humans two paralogous enzymes exist, both of them require mannose 1,6-bis-phosphate or glucose 1,6-bis-phosphate as activators, but only ...
Valentina Citro +5 more
doaj +4 more sources
Ginsenoside Rg1 can reverse fatigue behavior in CFS rats by regulating EGFR and affecting Taurine and Mannose 6-phosphate metabolism [PDF]
Frontiers in Pharmacology, 2023 Background: Chronic fatigue syndrome (CFS) is characterized by significant and persistent fatigue. Ginseng is a traditional anti-fatigue Chinese medicine with a long history in Asia, as demonstrated by clinical and experimental studies.
Chaofang Lei +11 more
doaj +2 more sources
Loss of the imprinted IGF2/cation-independent mannose 6-phosphate receptor results in fetal overgrowth and perinatal lethality. [PDF]
Genes & Development, 1994 Murine embryos that inherit a nonfunctional insulin-like growth factor-II/cation-independent mannose 6-phosphate receptor (Igf2r) gene from their fathers are viable and develop normally into adults.
M. Lau +5 more
openalex +2 more sources
Human immunodeficiency virus-1 uses the mannose-6-phosphate receptor to cross the blood-brain barrier. [PDF]
PLoS ONE, 2012 HIV-1 circulates both as free virus and within immune cells, with the level of free virus being predictive of clinical course. Both forms of HIV-1 cross the blood-brain barrier (BBB) and much progress has been made in understanding the mechanisms by ...
Shinya Dohgu +3 more
doaj +2 more sources
Fabry Cardiomyopathy: Myocardial Fibrosis, Inflammation, and Down‐Regulation of Mannose‐6‐Phosphate Receptors Cause Low Accessibility to Enzyme Replacement Therapy [PDF]
Journal of the American Heart Association: Cardiovascular and Cerebrovascular DiseaseBackground The clinical impact of enzyme replacement therapy on advanced Fabry disease cardiomyopathy appears to be limited. The pathologic mechanisms involved are still unclear.
Andrea Frustaci +5 more
doaj +2 more sources
Expression of Tailored α-N-Acetylglucosaminidase in Escherichia coli for Synthesizing Mannose-6-Phosphate on N-Linked Oligosaccharides of Lysosomal Enzymes [PDF]
BioengineeringLysosomal enzymes are synthesized as N-glycosylated glycoproteins with mannose-6-phosphate (M6P) moieties, which are responsible for their binding to M6P receptors and transporting to the lysosome. In the M6P biosynthetic pathway, a Man8GlcNAc2 glycoform
Yunsong Cao, Wei Wang
doaj +2 more sources
Targeted protein degradation through site-specific antibody conjugation with mannose 6-phosphate glycan [PDF]
mAbsRecent developments in targeted protein degradation have provided great opportunities to eliminating extracellular protein targets using potential therapies with unique mechanisms of action and pharmacology.
Kaori Mukai +11 more
doaj +2 more sources
Elevated mRNA expression and defective processing of cathepsin D in HeLa cells lacking the mannose 6‐phosphate pathway [PDF]
FEBS Open Bio, 2021 Disruption of the mannose 6‐phosphate (M‐6‐P) pathway in HeLa cells by inactivation of the GNPTAB gene, which encodes the α/β subunits of GlcNAc‐1‐phosphotransferase, results in missorting of newly synthesized lysosomal acid hydrolases to the cell ...
Lin Liu, Balraj Doray
doaj +2 more sources