IDUA Proteine (IDUA)

Iduronidase, alpha-L- Proteine (IDUA)
Auf finden Sie aktuell 12 Iduronidase, alpha-L- (IDUA) Proteine von 4 unterschiedlichen Herstellern. Zusätzlich bieten wir Ihnen IDUA Antikörper (61) und IDUA Kits (23) und viele weitere Produktgruppen zu diesem Protein an. Insgesamt sind aktuell 100 IDUA Produkte verfügbar.
6030426D08, alpha-L-iduronidase, fgfrl1, IDA, IDUA, MGC80842, MPS1, si:ch211-12e13.9, zgc:64084
alle Proteine anzeigen Gen GeneID UniProt
Ratte IDUA IDUA 360904  
IDUA 3425 P35475
IDUA 15932  

Weitere Synonyme anzeigen

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Weitere Proteine zu IDUA Interaktionspartnern

Zebrafish Iduronidase, alpha-L- (IDUA) Interaktionspartner

  1. In the absence of Fgfrl1a, larvae fail to express the transcription factor glial cells missing 2 (gcm2 (zeige GCM2 Proteine)), a gene necessary for cartilage and gill filament formation, in the ectodermal lining of the branchial arches.

Human Iduronidase, alpha-L- (IDUA) Interaktionspartner

  1. IDUA deletion mutation is associate with with mucopolysaccharidosis type I .

  2. Functional characterization of all the novel sequence variants identified in the study would be helpful to confirm the clinical significance and to determine the effect of these variations on the function of respective proteins (IDUA and IDS (zeige IDS Proteine))

  3. Molecular studies results unveiled the predominance of(Pro533Arg) IDUA variation in a series of 13 Algerian patients with Mucopolysaccharidosis Type I presented mainly with an attenuated phenotype.

  4. A new IDUA variant that alters the structure of the signal peptide associated with mucopolysaccharidosis type I is reported.

  5. Amino acid substitutions in alpha-L-iduronidase determine the severity of mucopolysaccharidosis type I.

  6. The alpha-L-iduronidase missense mutation causing L238Q substitution, when paired with a nonsense mutation, is associated with significant, late-onset brain disease.

  7. We conclude that this procedure for determining residual IDUA activity in fibroblasts of MPS I patients may be helpful to predict MPS I phenotype.

  8. The IDUA structures and biochemical analysis of the disease-relevant P533R mutation have enabled us to correlate the effects of mutations in IDUA to clinical phenotypes.

  9. Data show that alpha-l-iduronidase (hIDUA) enzyme activity was highly correlated with the N-glycan attached to N372.

  10. X-ray diffraction analysis of human alpha-L-iduronidase

Mouse (Murine) Iduronidase, alpha-L- (IDUA) Interaktionspartner

  1. This study demonstrated that the distribuation of bis (zeige BAG3 Proteine)(monoacylglycero)phosphate in brain of mucopolysaccharidosis 1 (Hurler) mouse.

  2. Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking alpha-l-iduronidase.

  3. Studies show that mouse Idua-W392X mutation is analogous to the human IDUA-W402X mutation commonly found in MPS I-H patients.

  4. Data show that late-stage erythroid cells, transduced with a tissue-specific lentiviral vector, can deliver alpha-L-iduronidase continuously and can correct the disease phenotype in both viscera and CNS of MPS I mice.

  5. The results indicate that Idua(-/-) mice present deficits in long-term memory for aversive training and reduced exploratory behavior.

  6. IDUA(-/-) mice from adolescence to maturity, exhibited locomotor and anxiety-like compulsive behaviors, spatial learning and memory, visual recognition and short-term non-associative memory retention.

IDUA Protein Überblick

Protein Überblick

This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I).

Alternative names and synonyms associated with IDUA

  • iduronidase, alpha-L- (idua)
  • iduronidase, alpha-L- (IDUA)
  • fibroblast growth factor receptor-like 1a (fgfrl1a)
  • fibroblast growth factor receptor-like 1 (FGFRL1)
  • alpha-l-iduronidase (AaeL_AAEL004402)
  • alpha-L-iduronidase (LOC100231378)
  • alpha-L-iduronidase (Idua)
  • iduronidase, alpha-L- (Idua)
  • 6030426D08 Protein
  • alpha-L-iduronidase Protein
  • fgfrl1 Protein
  • IDA Protein
  • IDUA Protein
  • MGC80842 Protein
  • MPS1 Protein
  • si:ch211-12e13.9 Protein
  • zgc:64084 Protein

Bezeichner auf Proteinebene für Iduronidase, alpha-L- Proteine (IDUA)

iduronidase, alpha-L- , alpha-L-iduronidase , fibroblast growth factor receptor-like 1 , alpha-l-iduronidase

446866 Xenopus laevis
511050 Bos taurus
567720 Danio rerio
100146682 Equus caballus
100560915 Anolis carolinensis
393347 Danio rerio
532327 Bos taurus
5564727 Aedes aegypti
100231378 Taeniopygia guttata
360904 Rattus norvegicus
100505382 Canis lupus familiaris
3425 Homo sapiens
15932 Mus musculus
427294 Gallus gallus
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