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Literature summary for 3.1.6.13 extracted from

  • Moro, E.; Tomanin, R.; Friso, A.; Modena, N.; Tiso, N.; Scarpa, M.; Argenton, F.
    A novel functional role of iduronate-2-sulfatase in zebrafish early development (2010), Matrix Biol., 29, 43-50.
    View publication on PubMed
Search for more literature for 3.1.6.13

Application

Application Comment Organism
medicine use of zebrafish as a novel tool to better understand lysosomal storage disorder pathogenesis Danio rerio

Cloned(Commentary)

Cloned (Comment) Organism
chimeric IDS-GFP cloned in a pCS2 vector under the control of the cytomegalovirus promoter. The resulting construct transfected into FU20 primary fibroblasts Danio rerio

Localization

Localization Comment Organism GeneOntology No. Textmining
cytoplasm
-
 Danio rerio 5737
-

Organism

Organism UniProt Comment Textmining
Danio rerio A0ST40
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain adult Danio rerio
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embryo IDS activity already detected at the 64-cell stage, and increases at 6.5 hours post fertilization, during the onset of gastrulation. A gradual decrease of enzymatic activity from 24 hours post fertilization to 5 dpf Danio rerio
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gut embryo and adult Danio rerio
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head embryo Danio rerio
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heart adult Danio rerio
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hindbrain embryo Danio rerio
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liver embryo Danio rerio
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mesenchyme vascular mesenchyme of the embryo Danio rerio
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midbrain embryo Danio rerio
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additional information embryonic otic vesicle, splanchnocranium and pectoral fin bud Danio rerio
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muscle adult Danio rerio
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ovary adult Danio rerio
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retina embryo Danio rerio
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tectum mesencephali embryo Danio rerio
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-iduronide-2-sulfate + H2O
-
 Danio rerio 4-methylumbelliferyl-alpha-iduronide + sulfate
-
 ?

Synonyms

Synonyms Comment Organism
IDS
-
 Danio rerio
iduronate sulfatase
-
 Danio rerio

Expression

Organism Comment Expression
Danio rerio knocking down with antisense morpholino oligos, significant decrease in the enzymatic activity after 24 hours post-morpholino injection in morphants (72.3% of the control). Inability of the morpholino to completely suppress the IDS activity due to maternally derived enzyme. Increased transforming growth factor beta signalling is tightly associated with downregulation of iduronate sulfatase function down
Danio rerio IDS is abundantly expressed during early developmental stages, IDS mRNAs is present already at the two-cell stage, pointing to a maternal origin of the transcripts. At later stages, ubiquitous expression from shield to 24 hours post fertilization. At 24 hours post fertilization a visible increased concentration of transcripts detectable in the head, particularly in the region surrounding the midbrain-hindbrain boundary. From the hatching phase (48 hours post fertilization), restriction of IDS transcription to the head, with a more intense staining in the retina, otic vesicles, the tectum and the vascular mesenchyme. More caudally, expression in the liver and pectoral fin buds. At 72 hours post fertilization IDS transcripts mainly present in two distinct areas, corresponding to the splanchnocranium and the gut, and a faint staining still in the otic vesicle. This expression pattern becomes more defined at 120 hours post fertilization, when the intensity of the hybridization signal in the gut and splanchnocranium. In the adult stage, IDS mRNAs in all the examined tissues up

General Information

General Information Comment Organism
malfunction downregulation of IDS may be responsible for severe early developmental defects, including a misshapen trunk and abnormal craniofacial cartilages, which is mediated by depauperation of sox10-expressing neural crest cell precursors, which is reverted through the administration of the recombinant enzyme Danio rerio
physiological function iduronate sulfatase plays a critical role during early vertebrate developments Danio rerio