Phenotype Image Detail

Image

Caption

Lysosomal storage pathology of liver and kidney. (A and A') Toluidine blue-stained Epon-embedded sections of liver from Arsg^tm1Tdi/Arsg^tm1Tdi (Arsg KO) mice (12 months old) reveal abnormal vacuoles in the peribiliary cytoplasm of hepatocytes (arrows) (S, sinusoid; LD, lipid droplet). (B and B') Endothelial cell of hepatic sinusoid contains cytoplasmic vacuoles with remnants of electron-dense material. (C and C') Cytoplasmic vacuoles in hepatocytes of mutant mice. (D and D') In kidney, the epithelium of the thick ascending limbs of Henle's loop displays cytoplasmic vacuoles that appear almost empty. (E-F') Cuprolinic blue incubation of liver (E and E') and kidney (F and F') sections reveals positive staining of inclusion bodies in hepatocytes (circle) and strong metachromatic GAG staining in liver sinusoidal cells (arrow) and in kidney thick ascending limbs (TAL) of Arsg-deficient mice. (G and H) Quantification of GAGs extracted from liver, brain, and kidney of Arsg-knockout and WT mice. The amounts of heparan sulfate (G) and chondroitin/dermatan sulfate (CS/DS) (H) were determined by LC/MS. Error bars indicate SD (n = 3).

Copyright

This image is from Kowalewski B, Proc Natl Acad Sci U S A 2012 Jun 26;109(26):10310-5. Copyright 2012 National Academy of Sciences, U.S.A. J:185592

Associated Alleles

Symbol	Name
Arsg^tm1Tdi	arylsulfatase G; targeted mutation 1, Thomas Dierks

Associated Genotypes

Allelic Composition	Genetic Background
Arsg^tm1Tdi/Arsg^tm1Tdi	involves: 129S2/SvPas * C57BL/6