Phenotype Image Detail

Image

Caption

Transverse sections of the optic nerve of control (A) and Arsa^tm1Gie/Arsa^tm1Gie mouse (B) stained with p-phenylenediamine. Myelin sheaths appear dark whereas axons and glial cells appear pale. Nonstained astrocytes appear as white patches, the number and size of which is clearly increased in the homozygous mouse. The cells feature thickened and elongated processes. Purkinje cells (C & D) were visualized by immunohistochemistry using and antibody to brain isoform of NO synthase. Cerebellar cortex of a control mouse is shown in C and that of a homozygous mutant mouse in D. Section of the inner ear of a control (E) and mutant (F) 11 month old mouse. Ganglion cells (G) and myelinated nerve fibers (NF) in the acoustic ganglion are severely reduced in the mutant mice. Some lipid-laden macrophages (MP) are observed. Arrows depict surviving neurons. Details of perisomatic (G & I) and periaxonal ( H & J) Schwann cells of control ( G & H) and homozygous mutant mice (I & J). In the mutant mouse, Schwann cells are swollen and filled with abnormal inclusions.

Copyright

This image is from Hess B, Proc Natl Acad Sci U S A 1996 Dec 10;93(25):14821-6. Copyright 1996 National Academy of Sciences, U.S.A. J:37978

Associated Alleles

Symbol	Name
Arsa^tm1Gie	arylsulfatase A; targeted mutation 1, Volkmar Gieselmann

Associated Genotypes

Allelic Composition	Genetic Background
Arsa^tm1Gie/Arsa^tm1Gie	involves: 129P2/OlaHsd * C57BL/6J