alpha-l-iduronidase

α-l-i·dur·on·id·ase

(ī'dūr-on'i-dās),
An enzyme that hydrolyzes terminal desulfated α-l-iduronic acid residues of dermatan sulfate and of heparan sulfate; a deficiency of this enzyme is associated with Hurler syndrome and Scheie syndrome.
References in periodicals archive ?
Mucopolysaccharidosis type I (MPS I) is a rare, lethal childhood genetic disease that affects the body's ability to produce IDUA (alpha-L-iduronidase), which is an essential enzyme that helps to break down long-chain sugars inside cells.
Engraftment and high alpha-L-iduronidase enzyme expression was seen in the first two patients with sufficient follow-up to assess these parameters.
MPS I and MPS II are caused by mutations in the genes encoding alpha-L-iduronidase (IDUA) and iduronate 2-sulfatase (IDS) enzymes, respectively.
She is missing a crucial enzyme called alpha-L-iduronidase, which breaks down toxins in the body.
Mucopoly saccharidosis type I: molecular characteristics of two novel alpha-L-iduronidase mutations in Tunisian patients.
The result is a lack of the enzyme alpha-L-iduronidase. Without it, sugar molecules accumulate in cells, causing irreversible tissue damage.
(Cambridge, MA) announced the issuance of a third United States patent involving the lysosomal enzyme alpha-L-iduronidase (IDUA), which is deficient in persons suffering from mucopolysaccharidosis type I (MPS I), also referred to as Hurler syndrome, Hurler-Scheie syndrome, or Scheie syndrome.
MPS I (also known as Hurler, Hurler-Scheie, and Scheie syndromes) is a life-threatening genetic disease caused by a deficiency of the enzyme alpha-L-iduronidase. This deficiency leads to the accumulation of complex carbohydrates (GAGs) in the lysosomes of cells, leading to the progressive dysfunction of cellular, tissue and organ systems.
MPS I is caused by mutations in the gene encoding the alpha-L-iduronidase (IDUA) enzyme.
Aldurazyme is a specific form of purified recombinant human alpha-L-iduronidase that is being investigated as an enzyme replacement therapy for MPS I, a life-threatening genetic disease for which no specific drug treatments currently exist.
MPS I and MPS II are caused by mutations in the genes encoding alpha-L-iduronidase and iduronate 2-sulfatase enzymes, respectively.
MPS I, which is a rare lysosomal storage disorder, is reportedly caused by mutations in the gene encoding the alpha-L-iduronidase (IDUA) enzyme.
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