Altered Sialylation of Glycoproteins and Its Impact on Disease Pathogenesis

2016 ◽  
pp. 158-174 ◽  
Keyword(s):  
Disease Pathogenesis

Insights into amyloid disease from fly models

2014 ◽  
Vol 56 ◽  
pp. 69-83 ◽  
Author(s):  
Ko-Fan Chen ◽  
Damian C. Crowther
Keyword(s):  
Drosophila Melanogaster ◽  
Neurodegenerative Disorders ◽  
Amyloid Β ◽  
Amyloid Β Peptide ◽  
Disease Pathogenesis ◽  
Amyloid Aggregates ◽  
Aβ Amyloid ◽  
Polypeptide Chains ◽  
Amyloid Diseases

The formation of amyloid aggregates is a feature of most, if not all, polypeptide chains. In vivo modelling of this process has been undertaken in the fruitfly Drosophila melanogaster with remarkable success. Models of both neurological and systemic amyloid diseases have been generated and have informed our understanding of disease pathogenesis in two main ways. First, the toxic amyloid species have been at least partially characterized, for example in the case of the Aβ (amyloid β-peptide) associated with Alzheimer's disease. Secondly, the genetic underpinning of model disease-linked phenotypes has been characterized for a number of neurodegenerative disorders. The current challenge is to integrate our understanding of disease-linked processes in the fly with our growing knowledge of human disease, for the benefit of patients.

Trans-sialidase Associated with Atherosclerosis: Defining the Identity of a Key Enzyme Involved in the Pathology

2019 ◽  
Vol 20 (9) ◽  
pp. 938-941
Author(s):  
Victor Y. Glanz ◽  
Veronika A. Myasoedova ◽  
Andrey V. Grechko ◽  
Alexander N. Orekhov
Keyword(s):  
Acid Metabolism ◽  
Research Subject ◽  
Disease Pathogenesis ◽  
Sialidase Activity ◽  
Ph Values ◽  
Ldl Particles ◽  
Optimum Ph ◽  
Key Enzyme ◽  
St6gal I

Atherosclerosis is associated with the increased trans-sialidase activity, which can be detected in the blood plasma of atherosclerosis patients. The likely involvement in the disease pathogenesis made this activity an interesting research subject and the enzyme that may perform such activity was isolated and characterized in terms of substrate specificity and enzymatic properties. It was found that the enzyme has distinct optimum pH values, and its activity was enhanced by the presence of Ca2+ ions. Most importantly, the enzyme was able to cause atherogenic modification of lowdensity lipoprotein (LDL) particles in vitro. However, the identity of the discovered enzyme remained to be defined. Currently, sialyltransferases, mainly ST6Gal I, are regarded as major contributors to sialic acid metabolism in human blood. In this mini-review, we discuss the possibility that atherosclerosis- associated trans-sialidase does, in fact, belong to the sialyltransferases family.

Airborne-induced experimental Bordetella bronchiseptica pneumonia in Strain 13 guineapigs

1987 ◽  
Vol 21 (3) ◽  
pp. 226-232 ◽  
Author(s):  
C. J. Trahan ◽  
E. H. Stephenson ◽  
J. W. Ezzell ◽  
W. C. Mitchell
Keyword(s):  
Weight Loss ◽  
Lethal Dose ◽  
Clinical Signs ◽  
Model System ◽  
Bordetella Bronchiseptica ◽  
Host Cells ◽  
Nasal Discharge ◽  
Disease Pathogenesis ◽  
Infectious Dose ◽  
Colony Forming Units

To evaluate the efficacy of a commercial bacterial vaccine in protecting Strain 13 guineapigs against fatal Bordetella bronchiseptica pneumonia, it was necessary to establish the infectivity and disease pathogenesis induced by virulent organisms. When guineapigs were exposed to small-particle aerosols of varying concentrations of virulent B. bronchiseptica, a spectrum of disease was produced that ranged from inapparent illness to fulminant bronchopneumonia. Clinical signs began by day 4 after exposure, and were evidenced by anorexia, weight loss, respiratory distress and serous to purulent nasal discharge. Pathological alterations were limited to the respiratory system. Moribund animals exhibited a suppurative necrotizing bronchopneumonia and necrotizing tracheitis. In animals that survived the challenge, the bacteria were eliminated from the lungs by day 28 but continued to persist in the laryngeal area and the trachea. The median infectious dose and the median lethal dose were estimated to be 4 colony-forming units (CFU) and 1314 CFU respectively. These data suggest that the guineapig will be a valuable model system in which to study interactions between Bordetella species and host cells as well as to evaluate potential B. bronchiseptica immunogens.

scholarly journals A Multi‐Step Model of Parkinson's Disease Pathogenesis

2021 ◽  
Author(s):  
Campbell Le Heron ◽  
Michael MacAskill ◽  
Deborah Mason ◽  
John Dalrymple‐Alford ◽  
Tim Anderson ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Disease Pathogenesis ◽  
Step Model

scholarly journals Mechanisms of Dinucleotide Repeat Instability in Escherichia coli

Genetics ◽  
2000 ◽  
Vol 154 (2) ◽  
pp. 533-542
Author(s):  
Marc Bichara ◽  
Isabelle Pinet ◽  
Sylvie Schumacher ◽  
Robert P P Fuchs
Keyword(s):  
Escherichia Coli ◽  
Mismatch Repair ◽  
Genetic Instability ◽  
Positional Cloning ◽  
Dinucleotide Repeats ◽  
Disease Pathogenesis ◽  
Complementary Sequence ◽  
Phylogenetic Studies ◽  
High Level

Abstract The high level of polymorphism of microsatellites has been used for a variety of purposes such as positional cloning of genes associated with diseases, forensic medicine, and phylogenetic studies. The discovery that microsatellites are associated with human diseases, not only as markers of risk but also directly in disease pathogenesis, has triggered a renewed interest in understanding the mechanism of their instability. In this work we have investigated the role of DNA replication, long patch mismatch repair, and transcription on the genetic instability of all possible combinations of dinucleotide repeats in Escherichia coli. We show that the (GpC) and (ApT) self-complementary sequence repeats are the most unstable and that the mode of replication plays an important role in their instability. We also found that long patch mismatch repair is involved in avoiding both short deletion and expansion events and also in instabilities resulting from the processing of bulges of 6 to 8 bp for the (GpT/ApC)- and (ApG/CpT)-containing repeats. For each dinucleotide sequence repeat, we propose models for instability that involve the possible participation of unusual secondary structures.

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