临床实践中的Star蛋白缺乏症:沙特阿拉伯的一系列病例

Eukaryotic cells can degrade proteins through several pathways. One of the most important amongst these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. A series of enzymes carry out the ubiquitination of the target proteins - E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...

Consuming animal-based products offers high-quality proteins that contain optimal levels and combinations of essential amino acids, crucial for tissue repair and growth. Foods like eggs, milk, fish, and most meats are a source of complete proteins. Legumes and cereals are abundant in proteins; however, they typically lack a full range of essential amino acids. As a result, they are considered incomplete protein sources. Some plant sources like soybeans, quinoa, and amaranth do contain complete...

Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...