关键词: Endocrinopathy Skeletal dysplasias neurological development sulfate

来  源:   DOI:10.1042/EBC20230097

Abstract:
Sulfate is an important anion as sulfonation is essential in modulation of several compounds, such as exogens, polysaccharide chains of proteoglycans, cholesterol or cholesterol derivatives and tyrosine residues of several proteins. Sulfonation requires the presence of both the sulfate donor 3\'-phosphoadenosine-5\'-phosphosulfate (PAPS) and a sulfotransferase. Genetic disorders affecting sulfonation, associated with skeletal abnormalities, impaired neurological development and endocrinopathies, demonstrate the importance of sulfate. Yet sulfate is not measured in clinical practice. This review addresses sulfate metabolism and consequences of sulfonation defects, how to measure sulfate and why we should measure sulfate more often.
摘要:
硫酸盐是一种重要的阴离子,因为磺化在几种化合物的调制中至关重要,比如外泌体,蛋白聚糖的多糖链,胆固醇或胆固醇衍生物和几种蛋白质的酪氨酸残基。磺化需要同时存在硫酸盐供体3'-磷酸腺苷-5'-磷酸硫酸盐(PAPS)和磺基转移酶。影响磺化的遗传疾病,与骨骼异常有关,受损的神经发育和内分泌疾病,证明硫酸盐的重要性。然而,在临床实践中无法测量硫酸盐。这篇综述涉及硫酸盐代谢和磺化缺陷的后果,如何测量硫酸盐以及为什么我们应该更频繁地测量硫酸盐。
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