Radiolabelled disaccharides for the assay of β-d-glucuronidase activity and the detection of mucopolysaccharidosis type VII

1982 ◽  
Vol 123 (3) ◽  
pp. 357-360 ◽  
Author(s):  
V.J. Muller ◽  
John J. Hopwood
Keyword(s):  
Mucopolysaccharidosis Type ◽  
Glucuronidase Activity

scholarly journals Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation

Blood ◽  
1991 ◽  
Vol 78 (11) ◽  
pp. 3081-3092 ◽  
Author(s):  
EH Birkenmeier ◽  
JE Barker ◽  
CA Vogler ◽  
JW Kyle ◽  
WS Sly ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Life Span ◽  
Marrow Transplantation ◽  
Mesangial Cells ◽  
Radiation Doses ◽  
Mucopolysaccharidosis Type ◽  
Glomerular Mesangial Cells ◽  
Glucuronidase Activity ◽  
Mps Vii

Abstract The gusmps/gusmps mouse has no beta-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic abnormalities are similar to those found in humans with severe MPS VII. Mutant mice are dysmorphic, dwarfed, and have a shortened life span. Pathologic findings include widespread lysosomal storage. To determine whether bone marrow transplantation (BMT) corrects these abnormalities, genetically identical mutant animals were given syngeneic bone marrow transplants using cells from +/+ mice. Initial experiments showed that levels of beta-glucuronidase activity in recipient tissues correlated with the amount of radiation administered before BMT. Two groups of mice given BMT therapy were observed for periods of 1 and 2 years, respectively. These mice were evaluated using a combination of clinical, biochemical, histochemical, and pathologic analyses. Spleen, liver, cornea, and glomerular mesangial cells showed essentially complete correction at all radiation doses. Storage was partially corrected in meninges and perivascular cells in brain, and in renal tubular epithelial cells at the higher radiation doses. Life span in BMT-treated animals was increased approximately three-fold, approaching that seen in normal mice after BMT. These results support the position that BMT has a place in the therapeutic regimen for MPS VII.

scholarly journals Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation

Blood ◽  
1991 ◽  
Vol 78 (11) ◽  
pp. 3081-3092 ◽  
Author(s):  
EH Birkenmeier ◽  
JE Barker ◽  
CA Vogler ◽  
JW Kyle ◽  
WS Sly ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Life Span ◽  
Marrow Transplantation ◽  
Mesangial Cells ◽  
Radiation Doses ◽  
Mucopolysaccharidosis Type ◽  
Glomerular Mesangial Cells ◽  
Glucuronidase Activity ◽  
Mps Vii

The gusmps/gusmps mouse has no beta-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic abnormalities are similar to those found in humans with severe MPS VII. Mutant mice are dysmorphic, dwarfed, and have a shortened life span. Pathologic findings include widespread lysosomal storage. To determine whether bone marrow transplantation (BMT) corrects these abnormalities, genetically identical mutant animals were given syngeneic bone marrow transplants using cells from +/+ mice. Initial experiments showed that levels of beta-glucuronidase activity in recipient tissues correlated with the amount of radiation administered before BMT. Two groups of mice given BMT therapy were observed for periods of 1 and 2 years, respectively. These mice were evaluated using a combination of clinical, biochemical, histochemical, and pathologic analyses. Spleen, liver, cornea, and glomerular mesangial cells showed essentially complete correction at all radiation doses. Storage was partially corrected in meninges and perivascular cells in brain, and in renal tubular epithelial cells at the higher radiation doses. Life span in BMT-treated animals was increased approximately three-fold, approaching that seen in normal mice after BMT. These results support the position that BMT has a place in the therapeutic regimen for MPS VII.

Characterization of the Defective ß-Glucuronidase Activity in Canine Mucopolysaccharidosis Type VII

Enzyme ◽  
1989 ◽  
Vol 42 (3) ◽  
pp. 174-180 ◽  
Author(s):  
Edward Schuchman ◽  
Tamitza Toroyan ◽  
Mark Haskins ◽  
Robert Desnick
Keyword(s):  
Mucopolysaccharidosis Type ◽  
Glucuronidase Activity

β-Glucuronidase activity in germ-free, monoassociated and conventional mice

2006 ◽  
Vol 18 (1) ◽  
Author(s):  
Maria E. Cardona ◽  
Elisabeth Norin ◽  
Tore Midtvedt
Keyword(s):  
Germ Free ◽  
Glucuronidase Activity

scholarly journals A New Paradigm in the Relationship between Melatonin and Breast Cancer: Gut Microbiota Identified as a Potential Regulatory Agent

Cancers ◽  
2021 ◽  
Vol 13 (13) ◽  
pp. 3141
Author(s):  
Aurora Laborda-Illanes ◽  
Lidia Sánchez-Alcoholado ◽  
Soukaina Boutriq ◽  
Isaac Plaza-Andrades ◽  
Jesús Peralta-Linero ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Gut Microbiota ◽  
Bacterial Population ◽  
Kynurenine Pathway ◽  
New Paradigm ◽  
Bacterial Composition ◽  
Glucuronidase Activity ◽  
The One ◽  
The Relationship ◽  
Melatonin Production

In this review we summarize a possible connection between gut microbiota, melatonin production, and breast cancer. An imbalance in gut bacterial population composition (dysbiosis), or changes in the production of melatonin (circadian disruption) alters estrogen levels. On the one hand, this may be due to the bacterial composition of estrobolome, since bacteria with β-glucuronidase activity favour estrogens in a deconjugated state, which may ultimately lead to pathologies, including breast cancer. On the other hand, it has been shown that these changes in intestinal microbiota stimulate the kynurenine pathway, moving tryptophan away from the melatonergic pathway, thereby reducing circulating melatonin levels. Due to the fact that melatonin has antiestrogenic properties, it affects active and inactive estrogen levels. These changes increase the risk of developing breast cancer. Additionally, melatonin stimulates the differentiation of preadipocytes into adipocytes, which have low estrogen levels due to the fact that adipocytes do not express aromatase. Consequently, melatonin also reduces the risk of breast cancer. However, more studies are needed to determine the relationship between microbiota, melatonin, and breast cancer, in addition to clinical trials to confirm the sensitizing effects of melatonin to chemotherapy and radiotherapy, and its ability to ameliorate or prevent the side effects of these therapies.

Blood and bone marrow findings in two pups with mucopolysaccharidosis type VII

2021 ◽  
Author(s):  
Erica Corda ◽  
Cheryl L. Swenson ◽  
Michael A. Scott ◽  
Dodd G. Sledge ◽  
John C. Fyfe
Keyword(s):  
Bone Marrow ◽  
Mucopolysaccharidosis Type
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