में अनुक्रमित
- यूरो पब
- गूगल ज्ञानी
उपयोगी कड़ियां
इस पृष्ठ को साझा करें
जर्नल फ़्लायर
एक्सेस जर्नल खोलें
- अभियांत्रिकी
- आनुवंशिकी एवं आण्विक जीवविज्ञान
- इम्यूनोलॉजी और माइक्रोबायोलॉजी
- औषधि विज्ञान
- कृषि और जलकृषि
- चिकित्सीय विज्ञान
- जीव रसायन
- जैव सूचना विज्ञान और सिस्टम जीवविज्ञान
- तंत्रिका विज्ञान और मनोविज्ञान
- नर्सिंग एवं स्वास्थ्य देखभाल
- नैदानिक विज्ञान
- पदार्थ विज्ञान
- पर्यावरण विज्ञान
- पशु चिकित्सा विज्ञान
- भोजन एवं पोषण
- रसायन विज्ञान
- व्यवसाय प्रबंधन
- सामान्य विज्ञान
अमूर्त
Treatment Strategies for Toxic Protein Progerin: Hutchinson- Gilford Progeria Syndrome
Sadaf Shamim
Hutchinson- Gilford Progeria Syndrome (HGPS; MIM 176670) is a rare fatal genetic disorder similar to a rapidly increasing age in symptoms. Aging is the main risk factor for heart diseases. It is becoming more prevalent in our societies. Hutchinson- Gilford Progeria Syndrome is an extremely infrequent inherited disease characterized by too early and fast aging immediately after being born. It a rare autosomal dominant genetic disorder, in 1 to 4 million births, for which there is no known cure. Children with HGPS succumb to myocardial infection and stroke in their teens and die of progressive vascular disease at an average age of 14 years. The average life of a child with HGPS is about 13 years. Some can die young with the disease and others may live longer, even for 20 years. The aim of this review is to understand the various aspects of disease special emphasis on pathophysiology, symptoms’, and recent trends in medicine and future treatments. Different treatment strategies could be used for the treatment of HGPS including ATP- based therapy, MB treatment, CRISPR Cas9. Vascular calcification results by pyrophosphate deficiency in children with HGPS. The mechanisms involved in vascular calcification in children with HGPS analyzed by the researches on mouse. ATP-based therapy could be used as treatment strategy for this devastating disease and other pyrophosphate related diseases. As HGPS is caused by C to T mutation in 11 exon, LMNA gene instead of activating cryptic site leaves amino acid codes unchanged that result in deletion of amino acids. These changes involves in different mechanisms like mitosis aberration, altered chromatin organization, transcriptional organization that causes nuclear abnormalities. Mitochondria is very intense organelle that plays very important role in our body, it also acts as key element in aging. Abnormal mitochondria can cause many damages by increasing levels of reactive oxygen species and altering DNA and protein structures. Mitochondrial abnormalities also reported in HGPS fibroblasts along with depletion of ATP and altered levels of ROS. HGPS fibroblasts of mouse are treated with methylene blue (MB), an antioxidant that normalizes the mitochondrial abnormalities and aging phenotypes in mouse.