mucopolysaccharide - définition. Qu'est-ce que mucopolysaccharide
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Qu'est-ce (qui) est mucopolysaccharide - définition

LONG UNBRANCHED POLYSACCHARIDES CONSISTING OF A REPEATING DISACCHARIDE UNIT
GAGs; Mucopolysaccharide; Mucopolysaccharides; Glycosaminoglycans; Glycoaminoglycans; GAGS; Glycoaminoglycan; ATC code M01AX12; ATCvet code QM01AX12

mucopolysaccharide         
[?mju:k??p?l?'sak?r??d]
¦ noun Biochemistry a substance occurring chiefly in connective tissue and consisting of complex polysaccharides containing amino groups.
Glycosaminoglycan galactosyltransferase         
CLASS OF ENZYMES
EC 2.4.1.74; UDP-alpha-D-galactose:glycosaminoglycan D-galactosyltransferase
In enzymology, a glycosaminoglycan galactosyltransferase () is an enzyme that catalyzes the chemical reaction
Mucopolysaccharidosis         
  • A child with an unspecified MPS disorder, showing characteristic facial features
  • Corneal clouding in a 30-year-old male with MPS-VI. Several other MPS disorders may also present with corneal clouding.
LYSOSOMAL STORAGE DISEASE THAT INVOLVES THE ACCUMULATION OF GLYCOSAMINOGLYCANS IN THE TISSUES AND THEIR EXCRETION IN THE URINE
Mucopolysaccharidoses; Mucopolysaccharide disease; Mucopolysaccharoidosis; Mucopolysaccharidosis type 3; Mucopolysaccharidosis type 4; Mucopolysaccharidosis type I Hurler syndrome; Mucopolysaccharidosis type I Hurler/Scheie syndrome; Mucopolysaccharidosis type I Scheie syndrome; Mucopolysaccharidosis type II Hunter syndrome- mild form; Mucopolysaccharidosis type II Hunter syndrome- severe form; Mucopolysaccharidosis type IV-A Morquio syndrome; Mucopolysaccharidosis type IV-B; Mucopolysaccharidosis type V; Scheie's syndrome; Scheie's Syndrome; Mucopolysaccharidosis affecting the skin; Mucopolysaccharidosis I S (Scheie)
Mucopolysaccharidoses are a group of metabolic disorders caused by the absence or malfunctioning of lysosomal enzymes needed to break down molecules called glycosaminoglycans (GAGs). These long chains of sugar carbohydrates occur within the cells that help build bone, cartilage, tendons, corneas, skin and connective tissue.

Wikipédia

Glycosaminoglycan

Glycosaminoglycans (GAGs) or mucopolysaccharides are long, linear polysaccharides consisting of repeating disaccharide units (i.e. two-sugar units). The repeating two-sugar unit consists of a uronic sugar and an amino sugar, except in the case of the sulfated glycosaminoglycan keratan, where, in place of the uronic sugar there is a galactose unit. GAGs are found in vertebrates, invertebrates and bacteria. Because GAGs are highly polar molecules and attract water; the body uses them as lubricants or shock absorbers.

Mucopolysaccharidoses are a group of metabolic disorders in which abnormal accumulations of glycosaminoglycans occur due to enzyme deficiencies.