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.
Department of Pathology, Sackler School of Medicine, Tel-Aviv University, Israel.
H
H
Weintraub
J
J
Alroy
R
R
DeGasperi
V
V
Goyal
E
E
Skutelsky
P G
PG
Pentchev
C D
CD
Warren
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