FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W1937494089> ?p ?o ?g. }

• 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.
• W1937494089 cites W1482589873 @default.
• W1937494089 cites W1487386905 @default.
• W1937494089 cites W1488029276 @default.
• W1937494089 cites W1496520030 @default.
• W1937494089 cites W1503392039 @default.
• W1937494089 cites W1507170913 @default.
• W1937494089 cites W1517883210 @default.
• W1937494089 cites W1520719553 @default.
• W1937494089 cites W1520780879 @default.
• W1937494089 cites W1523307634 @default.
• W1937494089 cites W1528293205 @default.
• W1937494089 cites W1529308973 @default.
• W1937494089 cites W1532138628 @default.
• W1937494089 cites W1539897505 @default.
• W1937494089 cites W1545930970 @default.
• W1937494089 cites W1548429343 @default.
• W1937494089 cites W1549719435 @default.
• W1937494089 cites W1549727212 @default.
• W1937494089 cites W1550282838 @default.
• W1937494089 cites W1554351458 @default.
• W1937494089 cites W1560452279 @default.
• W1937494089 cites W1561047920 @default.
• W1937494089 cites W1562872488 @default.
• W1937494089 cites W1568092407 @default.
• W1937494089 cites W1569256916 @default.
• W1937494089 cites W1572837727 @default.
• W1937494089 cites W157395064 @default.
• W1937494089 cites W1575992755 @default.
• W1937494089 cites W1576902195 @default.
• W1937494089 cites W1585193578 @default.
• W1937494089 cites W1590230123 @default.
• W1937494089 cites W1596311805 @default.
• W1937494089 cites W1604147470 @default.
• W1937494089 cites W1622692223 @default.
• W1937494089 cites W1628719417 @default.
• W1937494089 cites W1635366883 @default.
• W1937494089 cites W1672471316 @default.
• W1937494089 cites W170942874 @default.
• W1937494089 cites W1762877386 @default.
• W1937494089 cites W1764824114 @default.
• W1937494089 cites W1771766632 @default.
• W1937494089 cites W1866676984 @default.
• W1937494089 cites W1869006121 @default.
• W1937494089 cites W1895730431 @default.
• W1937494089 cites W1930709195 @default.
• W1937494089 cites W1932602652 @default.
• W1937494089 cites W1944766868 @default.
• W1937494089 cites W1949509566 @default.
• W1937494089 cites W1949693619 @default.
• W1937494089 cites W1951218128 @default.
• W1937494089 cites W1958074989 @default.
• W1937494089 cites W1963809496 @default.
• W1937494089 cites W1963961001 @default.
• W1937494089 cites W1964389840 @default.
• W1937494089 cites W1964653347 @default.
• W1937494089 cites W1964734663 @default.
• W1937494089 cites W1965359783 @default.
• W1937494089 cites W1966333400 @default.
• W1937494089 cites W1967321689 @default.
• W1937494089 cites W1967387547 @default.
• W1937494089 cites W1967811843 @default.
• W1937494089 cites W1968455741 @default.
• W1937494089 cites W1968666078 @default.
• W1937494089 cites W1968687601 @default.
• W1937494089 cites W1968957805 @default.
• W1937494089 cites W1970096704 @default.
• W1937494089 cites W1971019443 @default.
• W1937494089 cites W1971325550 @default.
• W1937494089 cites W1971846421 @default.
• W1937494089 cites W1972374716 @default.
• W1937494089 cites W1972557550 @default.
• W1937494089 cites W1973024069 @default.
• W1937494089 cites W1974231106 @default.
• W1937494089 cites W1974305316 @default.
• W1937494089 cites W1974739948 @default.
• W1937494089 cites W1975861627 @default.
• W1937494089 cites W1977575445 @default.
• W1937494089 cites W1978062178 @default.
• W1937494089 cites W1979087293 @default.
• W1937494089 cites W1979139698 @default.
• W1937494089 cites W1979952856 @default.
• W1937494089 cites W1980355423 @default.
• W1937494089 cites W1980617596 @default.
• W1937494089 cites W1980710017 @default.
• W1937494089 cites W1980741458 @default.
• W1937494089 cites W1981068090 @default.
• W1937494089 cites W1981850472 @default.
• W1937494089 cites W1982387497 @default.
• W1937494089 cites W1983532821 @default.
• W1937494089 cites W1984808800 @default.
• W1937494089 cites W1985456865 @default.
• W1937494089 cites W1985992934 @default.
• W1937494089 cites W1986684257 @default.
• W1937494089 cites W1987295447 @default.

• next