Investigation of the biological role of mouse acylneuraminyl hydrolase enzymes in the regulation of neuroinflammation için kapak resmi
Investigation of the biological role of mouse acylneuraminyl hydrolase enzymes in the regulation of neuroinflammation
Başlık:
Investigation of the biological role of mouse acylneuraminyl hydrolase enzymes in the regulation of neuroinflammation
Yazar:
Tabakacılar, Doğa, author.
Yazar Ek Girişi:
Fiziksel Tanımlama:
xi, 55 leaves: charts;+ 1 computer laser optical disc.
Özet:
Sialidosis is a lysosomal storage disorder, and it is inherited by autosomal recessive mutations in the Neuraminidase 1 (NEU1) gene. Neuraminidases or sialidases are catalytic enzymes that carry out the desialylation of glycoconjugates. Deficiencies of neuraminidases lead to the accumulation of sialoglycoconjugates in membranes of cells. Neuroinflammation and neurodegeneration are present in some lysosomal storage diseases such as Tay-Sachs. However, in the sialidosis mouse model, neuroinflammation was never studied. In this study, we investigated the effect of neuraminidase 1, neuraminidase 3, and their combined deficiency on neuroinflammation by using Neu1-/-, Neu3-/- knockout, and Neu1-/- Neu3-/- double knockout mouse models. Neu1-/- Neu3-/- knockout mouse model was smaller in comparison to its littermates and showed muscle weakness, tremoring, and 2-3 weeks of a lifetime. Some of the Neu1-/- Neu3-/- mice died prematurely. To unravel the pathology immunohistochemical, biological, and chromatographic techniques were used. The expression of inflammatory cytokines was altered in the Neu1-/-, Neu3-/-, and Neu1-/- Neu3-/- mice with respect to the brain section. Neu1-/- Neu3-/- mice showed generally the highest expression of cytokines in the cerebellum compared to the cortex. Neuronal loss was observed in the Neu1-/- Neu3-/- mice in the cortex, thalamus, and cerebellum. The most remarkable change was in the ganglioside expression pattern in the Neu1-/- Neu3-/- mice cortex. GD3 expression was present in the cortex of Neu1-/- Neu3-/- mice where expression of this ganglioside is related to neuroinflammation, neurodegenerative stimuli, autophagosome remodeling and programmed cell death. Keywords: Neuroinflammation, Sialidosis, Neuraminidase, Sialidase, Disease Pathology, NEU1, NEU3, Double Knockout, Transgenic Mouse Models.
Yazar Ek Girişi:
Tek Biçim Eser Adı:
Thesis (Master)--İzmir Institute of Technology:Molecular Biology and Genetics.

İzmir Institute of Technology:Molecular Biology and Genetics--Thesis (Master).
Elektronik Erişim:
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