Difference between revisions of "Part:BBa K5499002"

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Iduronate-2-sulfatase (IDS) is a lysosomal enzyme that splits the sulfate group in the C-2 positions from iduronic acid residues present in the mucopolysaccharides dermatan sulfate and heparan sulfate.   
 
Iduronate-2-sulfatase (IDS) is a lysosomal enzyme that splits the sulfate group in the C-2 positions from iduronic acid residues present in the mucopolysaccharides dermatan sulfate and heparan sulfate.   
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===Usage and Biology===
 
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A Deficiency in this enzyme results in the accumulation of these glycosaminoglycans in the lysosomes and subsequently leads to the development of Hunter syndrome(or mucopolysaccharidosis type 2 ) in humans.  The disorder is inherited as an X-linked recessive disease and has a broad spectrum of clinical phenotypes ranging from mild to severe forms.  This heterogeneity has been postulated to reflect the presence of different mutations in the IDS gene.
 
  
 
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===Usage and Biology===
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The IDS gene is located on the X chromosome (Xq28) and encodes the enzyme iduronate 2-sulfatase, which is involved in the breakdown of glycosaminoglycans (GAGs), specifically dermatan sulfate and heparan sulfate. Deficiency or malfunction of this enzyme, due to mutations in the IDS gene, leads to the accumulation of GAGs in tissues, causing Hunter syndrome. Hunter syndrome is an X-linked recessive disorder primarily affecting males and is characterized by a wide range of symptoms, including developmental delays, organ enlargement, and skeletal abnormalities. The enzyme functions within the lysosomes, and its malfunction leads to cellular dysfunction due to the accumulation of partially degraded GAGs. <br>
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The IDS gene is primarily used in medical research related to lysosomal storage disorders, specifically mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome. In therapeutic research, the IDS gene is a target for gene therapy approaches aimed at correcting the enzyme deficiency caused by mutations in the IDS gene. Recombinant IDS protein is also used for enzyme replacement therapy (ERT) to treat patients with Hunter syndrome. <br>
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This year, the NWU-CHINA-A team is dedicated to developing a new therapy that effectively addresses the shortcomings of existing treatments. Specifically, we aim to encapsulate IDS within engineered exosomes, modifying the exosomes to enable them to cross the blood-brain barrier. This approach seeks to overcome the limitations of ERT in alleviating the accumulation of glycosaminoglycans (GAGs) in the central nervous system.
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===Profile===
 
===Profile===
  
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Name: Iduronate-2-sulfatase (IDS)
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Name: IDS Gene <br>
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Base Pairs: 1650 bp <br>
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Base Pairs:1650 bp
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Origins: Human <br>
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Derived from the human genome, it encodes Iduronate-2-sulfatase (IDS), an enzyme that plays a crucial role in the metabolism of glycosaminoglycans (GAGs). <br>
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Origin: Homo sapiens, synthetic
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Properties: <br>
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The product of the IDS gene, Iduronate-2-sulfatase, is a lysosomal enzyme responsible for degrading specific sulfated sugar chains in glycosaminoglycans. <br>
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Properties: A kind of lysosomal enzyme that can degrade glycosaminoglycans（GAGs）
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Mutations in this gene can lead to Hunter Syndrome, an X-linked recessive disorder characterized by the abnormal accumulation of glycosaminoglycans. <br>
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The expression level of the IDS gene is closely related to the normal function of glycosaminoglycan metabolism, making it significant in gene therapy and biopharmaceutical applications.

Latest revision as of 09:08, 1 October 2024


Iduronate-2-sulfatase (IDS)

Iduronate-2-sulfatase (IDS) is a lysosomal enzyme that splits the sulfate group in the C-2 positions from iduronic acid residues present in the mucopolysaccharides dermatan sulfate and heparan sulfate.

Sequence and Features


Assembly Compatibility:
  • 10
    COMPATIBLE WITH RFC[10]
  • 12
    COMPATIBLE WITH RFC[12]
  • 21
    COMPATIBLE WITH RFC[21]
  • 23
    COMPATIBLE WITH RFC[23]
  • 25
    COMPATIBLE WITH RFC[25]
  • 1000
    COMPATIBLE WITH RFC[1000]


Usage and Biology

The IDS gene is located on the X chromosome (Xq28) and encodes the enzyme iduronate 2-sulfatase, which is involved in the breakdown of glycosaminoglycans (GAGs), specifically dermatan sulfate and heparan sulfate. Deficiency or malfunction of this enzyme, due to mutations in the IDS gene, leads to the accumulation of GAGs in tissues, causing Hunter syndrome. Hunter syndrome is an X-linked recessive disorder primarily affecting males and is characterized by a wide range of symptoms, including developmental delays, organ enlargement, and skeletal abnormalities. The enzyme functions within the lysosomes, and its malfunction leads to cellular dysfunction due to the accumulation of partially degraded GAGs.
The IDS gene is primarily used in medical research related to lysosomal storage disorders, specifically mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome. In therapeutic research, the IDS gene is a target for gene therapy approaches aimed at correcting the enzyme deficiency caused by mutations in the IDS gene. Recombinant IDS protein is also used for enzyme replacement therapy (ERT) to treat patients with Hunter syndrome.
This year, the NWU-CHINA-A team is dedicated to developing a new therapy that effectively addresses the shortcomings of existing treatments. Specifically, we aim to encapsulate IDS within engineered exosomes, modifying the exosomes to enable them to cross the blood-brain barrier. This approach seeks to overcome the limitations of ERT in alleviating the accumulation of glycosaminoglycans (GAGs) in the central nervous system.

Profile

Name: IDS Gene
Base Pairs: 1650 bp
Origins: Human
Derived from the human genome, it encodes Iduronate-2-sulfatase (IDS), an enzyme that plays a crucial role in the metabolism of glycosaminoglycans (GAGs).
Properties:
The product of the IDS gene, Iduronate-2-sulfatase, is a lysosomal enzyme responsible for degrading specific sulfated sugar chains in glycosaminoglycans.
Mutations in this gene can lead to Hunter Syndrome, an X-linked recessive disorder characterized by the abnormal accumulation of glycosaminoglycans.
The expression level of the IDS gene is closely related to the normal function of glycosaminoglycan metabolism, making it significant in gene therapy and biopharmaceutical applications.