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- W1937494089 abstract "Introduction: The basement membrane is a highly specialised extracellular matrix found underlying all endothelia and epithelia and surrounding many mesenchymal cells, in particular myocytes, peripheral nerves and adipocytes. Basement membranes have numerous physical and signalling functions that alter with the specific tissue type and stage of development. Nidogens are in mammals a family of two proteins nidogen -1 and 2. In vitro studies have indicated that the 150 kDa glycoprotein nidogen-1 may play a central role in the supramolecular organization of basement membranes through binding to a range of extracellular proteins. To elucidate their importance in the basement membrane, mice were generated with mutated alleles of the NID-1 gene. Ultrastructural analysis of kidney and skeletal muscle from mice lacking nidogen-1 failed to reveal differences in morphology and basement membrane structure. However, these mice show signs of neurological impairment with ataxia, especially of the hind limbs, and spontaneous seizure activity. Nidogen-2 staining in these animals is increased in certain basement membranes, particularly in cardiac and skeletal muscle, where it is normally found in scant amounts, suggesting that the loss of nidogen-1 may be compensated by nidogen-2. Mice have been generated lacking nidogen-1 and one or both alleles of NID-2. Mice lacking both nidogen-1 alleles and heterozygous of nidogen-2 show more severe neurological defects, than those lacking only nidogen -1. Aim of the project: To study the behavioural, electro-physiological, neurological and cellular aspects of the nidogen knockout mice (NID1 - -/NID2 + +) and (NID1 - -/NID2 + -), to gain a further insight into the function of this protein family. Results: The neurological defects were studied using rotarod tests, in vivo EEG recordings and in vitro hippocampal and neocortical field potential recordings analysing input/output relationships and short- and long term plasticity (paired-pulse behaviour and LTP). In vivo, the animals displayed massive functional deficits in the rotarod test and epileptiform discharges in EEG recordings. In vitro, in the hippocampus, 28% of the slices showed spontaneous, and another 33% evoked spontaneous epileptiform activity. Significant increases of the input/output ratio of synaptically evoked responses in CA1 and dentate gyrus, as well as of paired pulse accentuation, and loss of perforant path LTP was observed. By contrast, in the neocortex, the input/output ratio and paired-pulse accentuation were reduced. To augment the in vivo studies, mouse and human forms of nidogen-1 and-2 were cloned, recombinantly expressed and purified. Laminin was extracted from both control and nidogen knockout mice and the biochemical aspects of basement membrane deposition which seemed to be altered in the absence of nidogen was studied. A possible down-regulation of laminin and its receptors was investigated. Discussion: The results reveal the epileptic nature of the mice in the absence of nidogen-1. Alterations in synaptic plasticity and network function in the nidogen-1 null animals are indicative of a novel role for a nidogen-1, a protein found only in basement membranes. Also, it suggests that nidogen-1 is important for maintaining the structural integrity of basement membranes." @default.
- W1937494089 created "2016-06-24" @default.
- W1937494089 creator A5065242124 @default.
- W1937494089 date "2004-01-01" @default.
- W1937494089 modified "2023-09-22" @default.
- W1937494089 title "Neurological changes as a consequence of basement membrane defects- studies in the nidogen knockout mouse" @default.
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