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Phenotype Image Detail
Image
Caption A: Cross-section of stapes (St) with malleus (M) in wild type (WT) mouse. B: Higher magnification of rectangular area in A shows thin mucosal lining and regular bone surfaces. C: Analogous view to (A) in a Naglutm1Efn/Naglutm1Efn (MPS III) mouse at 30 weeks of age showing thickened mucosal layer on stapes and malleus (arrow). D: Enlarged view of rectangular area in C showing hyperplasia of mucosal cells (white arrows) and lysosomal storage in osteocytes (black arrowheads). Bone surface is pitted, indicating abnormal bone remodeling. E: Orthogonal view of stapes in a different mutant mouse showing otitis media with effusion. Note hyperplasia of mucosal cells (white arrow) and infiltrate with inflammatory cells (black arrow). F: Articulation point between incus and malleus in mutant mouse showing storage in chondrocytes (white arrowheads), hyperplasia and storage of mucosal cells (black arrow), and storage in inflammatory cells (white arrow). SA, Stapedial artery; F, Footplate of stapes; AL, Annular ligament at point of stapes insertion into cochlea; TM, Tympanic membrane.
Copyright This image is from Heldermon CD, PLoS ONE 2007;2(1):e772, and is displayed under the terms of the Creative Commons Attribution 4.0 International License. J:129390
Associated
Alleles
Symbol Name
Naglutm1Efn alpha-N-acetylglucosaminidase (Sanfilippo disease IIIB); targeted mutation 1, Elizabeth F Neufeld
Associated
Genotypes
Allelic Composition Genetic Background
Naglutm1Efn/Naglutm1Efn involves: 129S/SvEv * C57BL/6J

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
06/30/2020
MGI 6.15
The Jackson Laboratory