dermatan sulfate catabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the breakdown of dermatan sulfate, any of a group of glycosaminoglycans with repeats consisting of beta-(1,4)-linked L-iduronyl-beta-(1,3)-N-acetyl-D-galactosamine 4-sulfate units. [GOC:mah, ISBN:0198506732]

Dermatan sulfate catabolism is a complex biological process involved in the breakdown of dermatan sulfate, a glycosaminoglycan (GAG) found in various connective tissues, such as skin, cartilage, and blood vessels. This process is essential for maintaining tissue homeostasis, as it allows for the removal of damaged or aged dermatan sulfate, preventing its accumulation and contributing to the turnover of extracellular matrix components.

The catabolism of dermatan sulfate begins with the enzymatic degradation of the polysaccharide chain by a series of specific enzymes.

1. **Initial Steps:** The initial steps involve the action of **dermatan sulfate sulfatases** (DSS), which remove sulfate groups from the 6-O-sulfate groups of the N-acetylgalactosamine residues.
2. **Hydrolysis by Chondroitin Sulfatase:** Following desulfation, **chondroitin sulfatase** (CS) enzymes, specifically **CS-B**, hydrolyze the glycosidic bond between the N-acetylgalactosamine and glucuronic acid residues, resulting in the generation of disaccharides.
3. **Further Degradation:** These disaccharides can be further degraded by specific enzymes, such as **N-acetylgalactosamine-6-sulfatase** and **glucuronidase**, ultimately generating free monosaccharides that can be utilized by the cell.

**Regulation:** The catabolic process of dermatan sulfate is tightly regulated by various factors, including the expression levels of the involved enzymes and the presence of specific inhibitors. The degradation of dermatan sulfate is particularly important in certain diseases.

**Clinical Significance:** Defects in the enzymes involved in dermatan sulfate catabolism can lead to the accumulation of dermatan sulfate in various tissues, resulting in genetic disorders such as **mucopolysaccharidoses**, which are characterized by skeletal deformities, mental retardation, and other clinical manifestations.

In conclusion, the dermatan sulfate catabolic process is a crucial pathway involved in the breakdown and removal of dermatan sulfate from the body. This process ensures the proper turnover and maintenance of connective tissues, preventing the accumulation of damaged or aged GAGs and contributing to overall tissue homeostasis. Dysregulation of this process can lead to various diseases, emphasizing its vital role in health and disease.'
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Proteins (2)

Compounds (6)