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glycosaminoglycan metabolic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways involving glycosaminoglycans, any of a group of polysaccharides that contain amino sugars. [ISBN:0192800981]

Glycosaminoglycan metabolism is a complex biological process involving the synthesis, modification, and degradation of glycosaminoglycans (GAGs), a diverse family of linear, unbranched polysaccharides composed of repeating disaccharide units. These polysaccharides are found in various biological matrices, including extracellular matrix (ECM), cartilage, and connective tissues, playing crucial roles in cell signaling, structural support, and tissue hydration.

**Synthesis:**

GAG synthesis begins in the Golgi apparatus, where sugar monomers are added sequentially to a core protein, forming a proteoglycan. The core protein is typically a transmembrane protein or a protein associated with the ECM. Different types of GAGs are synthesized by specific glycosyltransferases, which add specific sugar units to the growing polysaccharide chain.

**Modification:**

After synthesis, GAGs undergo extensive modifications, including sulfation, epimerization, and acetylation. These modifications alter the charge and conformation of the GAG molecule, influencing its biological activity and interaction with other molecules.

**Degradation:**

GAGs are degraded by specific enzymes known as glycosidases, which cleave the glycosidic bonds between the sugar units. This degradation process occurs both intracellularly and extracellularly, with lysosomal enzymes playing a crucial role in intracellular degradation and extracellular matrix metalloproteinases (MMPs) contributing to extracellular degradation.

**Functions:**

* **Structural support:** GAGs contribute to the structural integrity of tissues, providing tensile strength and elasticity to the ECM.
* **Cell signaling:** GAGs bind to growth factors, cytokines, and other signaling molecules, regulating their activity and influencing cellular behavior.
* **Tissue hydration:** GAGs attract and retain water, contributing to tissue hydration and lubrication.
* **Regulation of inflammation:** GAGs modulate immune responses by interacting with immune cells and inflammatory mediators.

**Disorders:**

Defects in GAG metabolism can lead to a variety of genetic disorders, collectively known as mucopolysaccharidoses. These disorders are characterized by the accumulation of specific GAGs in various tissues, resulting in skeletal abnormalities, mental retardation, and other clinical manifestations.

**Therapeutic Applications:**

GAGs and their derivatives are being investigated for therapeutic applications in various fields, including:

* **Wound healing:** GAGs promote wound healing by stimulating cell migration and angiogenesis.
* **Osteoarthritis treatment:** GAGs can relieve joint pain and improve mobility in patients with osteoarthritis.
* **Cancer therapy:** GAGs have shown promise in inhibiting tumor growth and metastasis.

**Overall, glycosaminoglycan metabolism is a fundamental biological process that plays a vital role in maintaining tissue homeostasis, regulating cell signaling, and supporting diverse physiological functions.**'
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Proteins (2)

ProteinDefinitionTaxonomy
Beta-hexosaminidase subunit betaA beta-hexosaminidase subunit beta that is encoded in the genome of human. [PRO:DNx, UniProtKB:P07686]Homo sapiens (human)
Beta-hexosaminidase subunit alphaA beta-hexosaminidase subunit alpha that is encoded in the genome of human. [PRO:DNx, UniProtKB:P06865]Homo sapiens (human)

Compounds (6)

CompoundDefinitionClassesRoles
pyrimethamineMaloprim: contains above 2 cpdsaminopyrimidine;
monochlorobenzenes
antimalarial;
antiprotozoal drug;
EC 1.5.1.3 (dihydrofolate reductase) inhibitor
naphthalimidesNaphthalimides: Compounds with three fused rings that appear like a naphthalene fused to piperidone or like a benz(de)isoquinoline-1,3-dione (not to be confused with BENZYLISOQUINOLINES which have a methyl separating the naphthyl from the benzyl rings). Members are CYTOTOXINS.
2-acetamido-1,5-imino-1,2,5-trideoxy-d-glucitol2-acetamido-1,5-imino-1,2,5-trideoxy-D-glucitol: structure given in first source
2-(2-oxolanylmethyl)benzo[de]isoquinoline-1,3-dioneisoquinolines
n-acetylglucosamine thiazolineN-acetylglucosamine thiazoline: an analog of the oxazolinium bicyclic intermediate leading from N-acetylglucosamine to 1,6-anhydro-N-acetylmuramic acid
thiamet g