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Glycosaminoglycans (GAGs), also known as mucopolysaccharides, are long and linear polymers comprising of specific repeating disaccharides - the amino sugar that can be N-acetylglucosamine or N-acetylgalactosamine, and a uronic acid that is usually glucuronic acid or iduronic acid.
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Glycans, a class of complex heterogeneous molecules, can be covalently attached to proteins to form glycosylated proteins that regulate various physiological and pathological processes. Glycosylated proteins or glycoproteins comprise N-linked and O-linked oligosaccharides. O-glycosylation is the most common type of protein glycosylation. Here, glycans attach to the oxygen atom of the hydroxyl groups of Serine or Threonine residues. O-linked glycosylation occurs later in protein processing,...
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Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
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Protein glycosylation starts in the ER lumen and continues in the Golgi apparatus. Glycosyltransferases catalyze the addition of sugar molecules or glycosylation of proteins. Usually, these enzymes add sugars to the hydroxyl groups of selected serine or threonine residues to form O-linked glycans or the amino groups of asparagine residues to form N-linked glycans. Different positions on the same polypeptide chain can contain differently linked glycans.
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Biosynthesis of Polysaccharides01:26

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Polysaccharides such as glycogen and starch are synthesized from nucleoside diphosphate sugars, primarily uridine diphosphate glucose (UDPG) and adenosine diphosphate glucose (ADPG). These activated glucose donors act as key intermediates in carbohydrate metabolism and biosynthesis. UDPG primarily involves glycogen synthesis in animals and many bacteria, while ADPG plays a fundamental role in starch synthesis in plants and certain bacteria.UDPG is formed when glucose-1-phosphate reacts with...
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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Mucopolysaccharidosis: A broad review.

Ritu Nagpal1, Ram Bharos Goyal2, K Priyadarshini1

  • 1Cataract, Cornea & Refractive Surgery Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.

Indian Journal of Ophthalmology
|July 6, 2022
PubMed
Summary
This summary is machine-generated.

Mucopolysaccharidosis (MPS) is a genetic disorder causing glycosaminoglycan buildup. Treatments like enzyme replacement and keratoplasty improve quality of life, with gene therapy offering future promise.

Keywords:
Bilateral corneal cloudingHurler's syndromeMPSdiffuse corneal opacificationmucopolysaccharidosis

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Area of Science:

  • Genetics
  • Ophthalmology
  • Metabolic Disorders

Background:

  • Mucopolysaccharidosis (MPS) comprises seven types of genetic disorders.
  • Characterized by enzyme deficiencies leading to glycosaminoglycan (GAG) accumulation in lysosomes.
  • GAG accumulation affects multiple organ systems, including eyes, CNS, and skeletal structures.

Purpose of the Study:

  • To review the impact of MPS on visual function.
  • To discuss current and future treatment options for ocular manifestations of MPS.
  • To highlight the role of keratoplasty and emerging therapies for corneal clouding.

Main Methods:

  • Literature review of MPS, its ocular manifestations, and treatment strategies.
  • Analysis of current therapeutic modalities including bone marrow transplantation, enzyme replacement therapy, and keratoplasty.
  • Exploration of novel treatment approaches like gene therapy and substrate reduction therapy.

Main Results:

  • Visual impairment in MPS is common, caused by corneal clouding, retinopathy, and optic nerve issues.
  • Keratoplasty is a viable treatment for significant corneal clouding, but requires careful patient selection and counseling.
  • Enzyme replacement and bone marrow transplantation improve lifespan and quality of life.

Conclusions:

  • MPS significantly impacts vision, necessitating specialized ophthalmic care.
  • Keratoplasty offers visual rehabilitation for severe corneal opacity in MPS patients.
  • Emerging therapies like gene therapy show potential for reversing corneal clouding, potentially reducing the need for transplantation.