Cobalamin R binder as a possible model molecule for glycoprotein study in cystic fibrosis.

Abstract

The isoprotein pattern of semi-purified R binder (an acidic glycoprotein which binds cobalamin) from saliva and sera of 8 cystic fibrosis patients was compared to that of R binder from samples of 5 healthy children. In cases of cystic fibrosis, the mean isoelectric point of salivary R binder was increased from 3.78 up to 4.34 and its microheterogeneity was reduced. These significant physicochemical modifications were not observed with R binder from cystic fibrosis sera and they did not correlate with the beta-galactosidase, alpha-mannosidase, alpha-L-fucosidase nor neuraminidase activity of saliva. We propose the R binder as a model molecule to study the glycoprotein metabolism in cystic fibrosis since it contains 30-40% carbohydrate, is easily complexed with cyano[57Co]cobalamin and is present in most tissues and fluids of the human organism.

• Authors: J L Gueant, M Vidailhet, M Djalali, J c Michalski, J P Nicolas
• Journal: Clinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 143 Issue 3 Pg. 217-23 (Nov 30 1984) ISSN: 0009-8981 [Print] Netherlands
• PMID: 6437701 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Glycoproteins
• Transcobalamins
• Mannosidases
• Neuraminidase
• beta-Galactosidase
• alpha-Mannosidase
• alpha-L-Fucosidase

Topics

• Adolescent
• Child
• Child, Preschool
• Cystic Fibrosis (blood, enzymology, metabolism)
• Glycoproteins (metabolism)
• Humans
• Isoelectric Focusing
• Mannosidases (metabolism)
• Neuraminidase (metabolism)
• Protein Binding
• Saliva (metabolism)
• Transcobalamins (blood, metabolism)
• alpha-L-Fucosidase (metabolism)
• alpha-Mannosidase
• beta-Galactosidase (metabolism)