sodium-dodecyl-sulfate and Congenital-Disorders-of-Glycosylation

sodium-dodecyl-sulfate has been researched along with Congenital-Disorders-of-Glycosylation* in 1 studies

Other Studies

1 other study(ies) available for sodium-dodecyl-sulfate and Congenital-Disorders-of-Glycosylation

ArticleYear
Band 3 glycoprotein and glycophorin A from erythrocytes of children with congenital disorder of glycosylation type-Ia are underglycosylated.
    Proteomics, 2001, Volume: 1, Issue:2

    Band 3 and PAS-1 (a dimer of glycophorin A) from erythrocyte membranes of three children with congenital disorder of glycosylation, type Ia (CDG-Ia), aged 1 month, 3 years and 10 years respectively, were examined by a new technique that allowed determination of carbohydrate molar composition of glycoproteins separated by sodium dodecyl sulfate polyacrylamide gel electrophoresis. In CDG children a single N-glycan of band 3 glycoprotein was hypoglycosylated and its mannose content was normal or elevated. Glycophorin A which is the major carrier of erythrocyte sialic acid, was deficient in N-acetylgalactosamine, and sialic acid residues. This finding indicated a partial unglycosylation of O-glycans in glycophorin A. In keeping with the results of PAS-1 analysis, total sialic acid in erythrocyte membranes from CDG children was reduced to 40-56% of normal values. A possible molecular mechanism of hypo- and unglycosylation of band 3 and glycophorin A, respectively, in CDG is discussed.

    Topics: Anion Exchange Protein 1, Erythrocyte; Carbohydrate Sequence; Carbohydrates; Case-Control Studies; Child; Child, Preschool; Congenital Disorders of Glycosylation; Electrophoresis, Polyacrylamide Gel; Erythrocyte Membrane; Glycophorins; Glycosylation; Humans; Infant; Molecular Sequence Data; N-Acetylneuraminic Acid; Proteome; Sodium Dodecyl Sulfate

2001
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