Phenotype Image Detail

Image

Caption

A: Liver of a Naglu^tm1Efn/Naglu^tm1Efn mouse at 3 months of age. Note the very large vacuoles in the Kupffer cell (arrowhead) and much smaller vacuoles in the hepatocyte (arrows), both containing fine granular material. B: Podocyte of the homozygous mouse at 6 months of age, showing vacuoles with fine granular material (arrow). C: Macrophage-like cells with vacuolated cytoplasm in the caudatoputamen of the homozygous mouse at 6 months old (hematoxylin/eosin stain). Arrows point to individual cells, whereas arrowhead points to a cluster. D: Neuron of dentate nucleus of cerebellum with large inclusions stained light blue (arrow) in the homozygous mouse (left) and absence of such inclusions in cell from same area of a control mouse (right) both at 6 months of age (Kluver-Barrera luxol fast blue stain). E: Purkinje cell from 6 month old homozygous mouse. Note the large inclusion containing lamellar as well as coarse granular material; arrowheads point to edge of the inclusion. F: Pericyte from a homozygous mouse at 6 months old. A very large pleiomorphic inclusion (arrowhead) is present in addition to inclusions containing granular material (arrow). Red & yellow bars: 1um

Copyright

This image is from Li HH, Proc Natl Acad Sci U S A 1999 Dec 7;96(25):14505-10. Copyright 1999 National Academy of Sciences, U.S.A. J:58950

Associated Alleles

Symbol	Name
Naglu^tm1Efn	alpha-N-acetylglucosaminidase (Sanfilippo disease IIIB); targeted mutation 1, Elizabeth F Neufeld

Associated Genotypes

Allelic Composition	Genetic Background
Naglu^tm1Efn/Naglu^tm1Efn	either: (involves: 129S/SvEv * C57BL/6) or (involves: C57BL/6)