Phenotype Image Detail

Image

Caption

Electron micrographic analysis of Gusb^mps/Gusb^mps Tg(GUSB)4Sly/Tg(GUSB)4Sly (transgenic) and Gusb^mps/Gusb^mps (mutant) mice. Visceral epithelial cells in a glomerulus from a 3 month old mutant male mouse (A) are distended by enlarged lysosomes (arrow) containing fine fibrillar material and small fragments of membranous debris. In contrast, a 2 month old male transgenic mutant mouse (B) had no lysosomal distension in glomerular podocytes (arrow). A splenic vascular space from a newborn mutant pup (C) is lined by cells distorted by numerous expanded lysosomes. Much of the storage material was lost in processing, and only small membranous fragments (arrow) remain. A 2 month old male transgenic mutant mouse (D) had no significant vacuolar storage in vascular lining cells (arrow). Magnification: A, x3100; B, x2850; C, X2250; D, x3850. Scalebars: 5um. uranyl acetate-lead citrate.

Copyright

This image is from Kyle JW, Proc Natl Acad Sci U S A 1990 May;87(10):3914-8. Copyright 1990 National Academy of Sciences, U.S.A. J:21256

Associated Alleles

Symbol	Name
Gusb^mps	glucuronidase, beta; beta glucuronidase, mucopolysaccharidosis VII
Tg(GUSB)4Sly	transgene insertion 4, William S Sly

Associated Genotypes

Allelic Composition	Genetic Background
Gusb^mps/Gusb^mps	B6.Cg-Gusb^mps/BrkJ
Gusb^mps/Gusb^mps Tg(GUSB)4Sly/Tg(GUSB)4Sly	involves: C57BL/6By * C57BL/6J * LT/Sv * SJL/J