Alpha-L-iduronidase (Q21496504)

http://www.ebi.ac.uk/interpro/protein/P48441

glycoside hydrolase superfamily

1 reference

stated in

InterPro Release 71.0

http://www.ebi.ac.uk/interpro/protein/P48441

Glycoside hydrolase, family 39

1 reference

stated in

InterPro Release 71.0

http://www.ebi.ac.uk/interpro/protein/P48441

1 reference

20 March 2016

language of work or name

1 reference

20 March 2016

language of work or name

English

molecular function

L-iduronidase activity

4 references

24 April 2018

http://www.ebi.ac.uk/QuickGO/annotations?protein=P48441&geneProductId=UniProtKB:P48441

24 April 2018

http://www.ebi.ac.uk/QuickGO/annotations?protein=P48441&geneProductId=UniProtKB:P48441

24 April 2018

http://www.ebi.ac.uk/QuickGO/annotations?protein=P48441&geneProductId=UniProtKB:P48441

24 April 2018

http://www.ebi.ac.uk/QuickGO/annotations?protein=P48441&geneProductId=UniProtKB:P48441

hydrolase activity, hydrolyzing O-glycosyl compounds

IBA

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

hydrolase activity, acting on glycosyl bonds

IEA

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

signaling receptor binding

IEA

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

ISO

L-iduronidase activity

9 references

14 February 2019

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

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

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

2 references

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

skeletal system morphogenesis

1 reference

14 February 2019

Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

glycosaminoglycan catabolic process

1 reference

24 April 2018

http://www.ebi.ac.uk/QuickGO/annotations?protein=P48441&geneProductId=UniProtKB:P48441

carbohydrate metabolic process

ISO

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

glycosaminoglycan catabolic process

IEA

7 references

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

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

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice

adult walking behavior

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

adult locomotory behavior

1 reference

14 February 2019

Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

extracellular matrix organization

1 reference

14 February 2019

Extracellular matrix disruption is an early event in the pathogenesis of skeletal disease in mucopolysaccharidosis I.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

dermatan sulfate catabolic process

5 references

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

heparin catabolic process

IDA

5 references

14 February 2019

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

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

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

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

positive regulation of bone resorption

IDA

1 reference

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

1 reference

14 February 2019

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

negative regulation of cartilage development

1 reference

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

negative regulation of secretion of lysosomal enzymes

2 references

14 February 2019

Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

14 February 2019

Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

positive regulation of collagen catabolic process

IDA

1 reference

14 February 2019

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities

cartilage homeostasis

1 reference

14 February 2019

http://www.ebi.ac.uk/QuickGO/annotations?geneProductId=UniProtKB:P48441