Storage of glycoprotein in NCTR-Balb/C mouse. Lectin histochemistry, and biochemical studies.
A strain of Balb/C mice carrying a lysosomal storage disorder exhibits metabolic and phenotypic abnormalities similar to patients with sphingomyelin-cholesterol lipidoses type II (i.e., Niemann-Pick C and D). Their foamy cells, which belong to the reticuloendothelial system, stained intensely by periodate-Schiff (PAS) reagent and were resistant to predigestion with diastase. To identify the chemical nature of the PAS-positive storage material, we applied lectin histochemistry and biochemical methods. Paraffin embedded sections, and delipidated frozen tissue sections, were treated with biotinylated lectins and localized with avidin-biotin-peroxidase complex. Araldite-embedded semithin sections were incubated with biotinylated lectins followed by avidin-gold and were enhanced with silver. By both histochemical methods the affected foamy cells stained positively as follows: Concanavalia ensiformis agglutinin, Datura stramonium agglutinin, Griffonia simplicifolia-I, Lens culinaris agglutinin, peanut agglutinin, Ricinus communis agglutinin-I, wheat germ agglutinin (WGA), and succinylated-WGA. Biochemical analysis of liver extracts complemented the histochemical data and demonstrated accumulation of glycoproteins containing polylactosaminoglycans in affected mice. Our findings indicate that the storage material in NCTR-Balb/C mice is heterogeneous. The lipids that are extracted by organic solvents during the histologic preparations mask the occurrence of polylactosaminoglycan containing glycoproteins in native frozen sections.
Weintraub
H
H
Department of Pathology, Sackler School of Medicine, Tel-Aviv University, Israel.
Alroy
J
J
DeGasperi
R
R
Goyal
V
V
Skutelsky
E
E
Pentchev
P G
PG
Warren
C D
CD
D000818:Animals; D006020:Glycopeptides / Q000032:analysis; D006023:Glycoproteins / Q000378:metabolism*; D006651:Histocytochemistry; D037102:Lectins; D008099:Liver / Q000378:metabolism; D051379:Mice; D008817:Mice, Mutant Strains; D009844:Oligosaccharides / Q000032:analysis
D006020:Glycopeptides; D006023:Glycoproteins; D037102:Lectins; D009844:Oligosaccharides