Abnormal basement membrane in the inner ear and the kidney of the Mpv17-/- mouse strain: ultrastructural and immunohistochemical investigations

Gottesberge, A. M. Meyer zum; Felix, Heidi

doi:10.1007/s00418-005-0027-7

Cited by 27 publications

(19 citation statements)

References 60 publications

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“…Mammalian BMs are intertwined networks of extracellular matrix (ECM) molecules consisting of polymeric laminin and type IV collagen bridged by non-covalent interactions with nidogens and proteoglycans (Kalluri 2003). The mammalian inner ear is rich in BMs, and alterations of the BMs are proposed to mediate the pathology of hearing disorders including Alport’s syndrome and presbycusis (Cosgrove et al 1998; Gratton et al 2005; Meyer zum Gottesberge and Felix 2005; Sakaguchi et al 1997; Zehnder et al 2005). …”

Section: Introductionmentioning

confidence: 99%

Cochlin expression in vestibular endorgans obtained from patients with Meniere’s disease

et al. 2012

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The distribution of cochlin and its associated basement membrane proteins (collagen IV, collagen II, laminin-β2, and nidogen-1) was evaluated in the vestibular endorgans of subjects with Meniere’s disease and compared to normal specimens. Cochlin mRNA expression in vestibular endorgans from Meniere’s disease specimens was also investigated. Specimens were obtained from patients with Meniere’s disease who underwent ablative labyrinthectomy. Control specimens were obtained from both autopsy specimens with documented normal audiovestibular function and from patients undergoing labyrinthectomy for acoustic neuroma excision. In the normal control specimens, cochlin immunoreactivity was found evenly distributed in the stroma of the cristae ampullaris and maculae of the utricle. In Meniere’s specimens, cochlin immunoreactivity was markedly increased, which was associated with an increase in cochlin mRNA expression on real-time RT-PCR. Collagen IV and laminin-β2 immunoreactivity was significantly decreased in Meniere’s specimens. Nidogen-1 and collagen II immunoreactivity were unchanged in Meniere’s specimens when compared with normal specimens. Cochlin upregulation has been implicated in the hereditary audiovestibulopathy, DFNA9. The increased expression of cochlin and decreased expression of collagen IV and laminin in Meniere’s disease are suggestive that overexpression of cochlin may contribute to the dysfunctional inner ear homeostasis seen in Meniere’s disease.

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Section: Introductionmentioning

confidence: 99%

Cochlin expression in vestibular endorgans obtained from patients with Meniere’s disease

et al. 2012

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“…An extensive network of BMs is also associated with the spiral ganglion cells and their associated axons as well as with the vessels of the inner ear. 2,6 In the cochlea, three distinct types of epithelia surround the endolymphatic compartment: the sensory epithelium (eg organ of Corti), the ion transporting epithelium (eg the stria vascularis), and a third, unspecialized epithelial layer (reviewed by Raphael and Altschuler 7 ). While the organ of Corti is composed of various types of sensory hair cells and accessory supporting cells, the stria vascularis is a complex tissue composed of several different cell types.…”

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confidence: 99%

Inner ear defects and hearing loss in mice lacking the collagen receptor DDR1

Gottesberge

Groß

Becker-Lendzian

et al. 2008

Laboratory Investigation

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Discoidin domain receptor 1 (DDR1) is a tyrosine kinase receptor that is activated by native collagen. The physiological functions of DDR1 include matrix homeostasis and cell growth, adhesion, branching, and migration, but the specific role of DDR1 in the development and function of the inner ear has not been analyzed. Here, we show that deletion of the DDR1 gene in mouse is associated with a severe decrease in auditory function and substantial structural alterations in the inner ear. Immunohistochemical analysis demonstrated DDR1 expression in several locations in the cochlea, mostly associated with basement membrane and fibrillar collagens; in particular in basal cells of the stria vascularis, type III fibrocytes, and cells lining the basilar membrane of the organ of Corti. In the stria vascularis, loss of DDR1 function resulted in altered morphology of the basal cells and accumulation of electron-dense matrix within the strial epithelial layer in conjunction with a focal and progressive deterioration of strial cells. Cell types in proximity to the basilar membrane, such as Claudius', inner and outer sulcus cells, also showed marked ultrastructural alterations. Changes in the organ of Corti, such as deterioration of the supporting cells, specifically the outer hair cells, Deiters', Hensen's and bordering cells, are likely to interfere with mechanical properties of the organ and may be responsible for the hearing loss observed in DDR1-null mice. These findings may also have relevance to the role of DDR1 in other disease processes, for example, those affecting the kidney.

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“…In mice deficient in the peroxisomal Mpv17-protein, which appears to be involved in the homeostasis of reactive oxygen species, apoptosis, probably due to an imbalance in reactive oxygen species has been reported (Meyer Zum Gottesberge and Felix 2005).…”

Section: Programmed Cell Death Through Apoptosismentioning

confidence: 96%

“…Lipid peroxidation-detoxifying enzymes such as aldehyde reductase were analyzed by Iuchi et al (2004) who were able to demonstrate the presence of a defense mechanism in the male genital tract by the co-localization of aldehyde reductase and lipid peroxidation products. The consequences of the loss of the function of the peroxisomal Mpv17-protein and associated imbalanced radical oxygen species (ROS) homeostasis for the basement membrane structure in the inner ear of Mpv 17 -/-mice was investigated by Meyer Zum Gottesberge and Felix (2005). The basement membrane (BM) of the stria vascularis of the inner ear (and of renal glomeruli) was affected in early stages of the disease and resulted in its excessive enlargement.…”

Section: Histochemical Techniques Applied In Disease Researchmentioning

confidence: 99%

Recent progress in histochemistry and cell biology: the state of the art 2005

Taatjes

Roth

2005

Histochem Cell Biol

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Advances in the field of histochemistry, a multidisciplinary area including the detection, localization and functional characterization of molecules in single cells and complex tissues, often drives the attainment of new knowledge in the broadly defined discipline of cell biology. These two disciplines, histochemistry and cell biology, have been joined in this journal to facilitate the flow of information with celerity from technical advancement in histochemical procedures, to their utilization in experimental models. This review summarizes advancements in these fields during the past year.

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Abnormal basement membrane in the inner ear and the kidney of the Mpv17-/- mouse strain: ultrastructural and immunohistochemical investigations

Cited by 27 publications

References 60 publications

Cochlin expression in vestibular endorgans obtained from patients with Meniere’s disease

Cochlin expression in vestibular endorgans obtained from patients with Meniere’s disease

Inner ear defects and hearing loss in mice lacking the collagen receptor DDR1

Recent progress in histochemistry and cell biology: the state of the art 2005

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