期刊
BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
卷 1865, 期 8, 页码 -出版社
ELSEVIER
DOI: 10.1016/j.bbagen.2021.129898
关键词
Galactose; Galactosemia; Congenital disorders of glycosylation; Lysosomal disorders; Collagen glycosylation; Keratan sulfate
资金
- Prinses Beatrix Spierfonds foundation [W.OR15]
- Netherlands Organization for Scientific Research [VIDI] [91,713,359]
- Stofwisselkracht
Galactose is essential for cellular metabolism and glycosylation, playing important roles in energy production, storage, and various biological functions. Defects in galactosylation and galactose homeostasis can lead to a range of pathogenic conditions, underscoring the significance of galactose metabolism in human health.
Galactose is an essential carbohydrate for cellular metabolism, as it contributes to energy production and storage in several human tissues while also being a precursor for glycosylation. Galactosylated glycoconjugates, such as glycoproteins, keratan sulfate-containing proteoglycans and glycolipids, exert a plethora of biological functions, including structural support, cellular adhesion, intracellular signaling and many more. The biological relevance of galactose is further entailed by the number of pathogenic conditions consequent to defects in galactosylation and galactose homeostasis. The growing number of rare congenital disorders involving galactose along with its recent therapeutical applications are drawing increasing attention to galactose metabolism. In this review, we aim to draw a comprehensive overview of the biological functions of galactose in human cells, including its metabolism and its role in glycosylation, and to provide a systematic description of all known congenital metabolic disorders resulting from alterations of its homeostasis.
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