ライフサイエンスコーパス: dysmyelination

1 (LPS) administration to mothers and eventual dysmyelination.

2 ropathy mice that cannot be accounted for by dysmyelination.

3 posed regarding how the lack of QKI leads to dysmyelination.

4 everity from early embryonic death to viable dysmyelination.

5 Loss of mature OLs results in severe CNS dysmyelination.

6 ity, and its absence leads to late-onset CNS dysmyelination.

7 layed oligodendrocyte maturation, leading to dysmyelination.

8 Loss of OPTN led to progressive dysmyelination and axonal degeneration through engagemen

9 Failure in this interaction causes dysmyelination and axonal degeneration.

10 rized by severe central nervous system (CNS) dysmyelination and demyelination, a conspicuous action t

11 m in the MD rat at this age showed extensive dysmyelination and downregulation of NMDA and to a lesse

12 Thus, Mtmr13(-/-) mice show both the initial dysmyelination and later degenerative pathology of CMT4B

13 phological studies show evidence for primary dysmyelination and myelin instability in affected animal

14 avan disease, neuroimaging shows early-onset dysmyelination and progressive brain atrophy.

15 lin basic protein that results in severe CNS dysmyelination and subsequent demyelination during devel

16 The consequences of dysmyelination are poorly understood and vary widely in

17 deficient (md) rats, which have profound CNS dysmyelination associated with oligodendrocyte cell deat

18 lipid protein and is characterized by severe dysmyelination attributable to oligodendrocyte death.

19 stribution of other myelin proteins, causing dysmyelination, axonal damage, or both.

20 integrity of macromolecules such as myelin ('dysmyelination') can be studied by applying multiple mod

21 rs persist after oligodendrocyte cell death; dysmyelination does not alter the expression of differen

22 mpanied by peripheral neuropathy and central dysmyelination has been recognized recently in associati

23 ng with life-threatening cerebral oedema and dysmyelination in affected individuals.

24 aracterized by severe central nervous system dysmyelination in affected males, and myelin mosaicism i

25 in oligodendrocytes results in differential dysmyelination in specific areas of the CNS, with the gr

26 hibit delayed axon myelination and increased dysmyelination in the central nervous system.

27 normal numbers of Schwann cells and profound dysmyelination in the central nervous system.

28 in muscle degeneration and peripheral nerve dysmyelination in the dy2J dystrophic mouse.

29 d reveal key insights into the mechanisms of dysmyelination in the quakingviable mutant.

30 Our findings suggest that dysmyelination in visual processing regions is present i

31 Dysmyelination is evident in Mtmr13-deficient nerves at

32 We conclude that: 1) dysmyelination is less severe in the shk PNS than in the

33 In addition, dysmyelination led to a decreased synaptic quantal conte

34 St3gal2/3 double-null mice displayed dysmyelination marked by a 40% reduction in major myelin

35 associated with both peripheral and central dysmyelination may affect pathology through a dominant-n

36 In conclusion, AN dysmyelination may be of fundamental importance in audit

37 n in Schwann cells leads to hypermyelination/dysmyelination, mimicking some features present in neuro

38 quaking (qk), one of the classical mouse dysmyelination mutants, is defective for the expression

39 Quakingviable (qk(v)) is a well known dysmyelination mutation.

40 myelin-deficient rats, which have severe CNS dysmyelination, node-like clusters of Kv3.1b and Na+ cha

41 ical disorders, in which complete or partial dysmyelination occurs in either the central nervous syst

42 analysis of the sciatic nerve showed de- and dysmyelination of fibers, with massive outfoldings and a

43 We documented that Msh2 deficiency causes dysmyelination of the axonal projections in the corpus c

44 MD) is an X-linked disorder characterized by dysmyelination of the central nervous system (CNS) cause

45 ed genetic disorder that is characterized by dysmyelination of the central nervous system resulting p

46 sive neurological mouse mutation with severe dysmyelination of the CNS and spermiogenesis failure.

47 ng Evans shaker (les) rat that causes severe dysmyelination of the CNS.

48 ults suggest that the primary abnormality is dysmyelination of the optic nerve in early development.

49 n events along the nerve trunk but developed dysmyelination of the preterminal segment associated wit

50 Previous observations of the effect of dysmyelination or demyelination on axonal survival in th

51 le for the severe reduction of MBPs in qk(v) dysmyelination, presumably because of the lack of intera

52 the white matter of FCD IIb was related to a dysmyelination process associated with severe fiber loss

53 attenuated injury-induced ROS production and dysmyelination processes of peripheral nerves.

54 quakingviable mutants, which display severe dysmyelination, QKI-6 and QKI-7 are absent exclusively f

55 cks large diameter axons, suggesting primary dysmyelination, rather than a demyelinating process.

56 spike upstroke and downstroke kinetics, but dysmyelination reduces the after-depolarization and enha

57 h we can begin to understand the early onset dysmyelination seen in patients with R98C and similar mu

58 also reveal significantly less demyelination/dysmyelination than in the null alleles.

59 fatal, connatal disease, including extensive dysmyelination, tremors, ataxia, and death at approximat

60 n and blood-brain barrier disruption without dysmyelination were the major neuropathological alterati

61 o normal birth weight, growth failure, brain dysmyelination with calcium deposits, cutaneous photosen

62 o-normal birth weight; growth failure; brain dysmyelination with calcium deposits; cutaneous photosen

63 w, GJC2 mutations were only thought to cause dysmyelination, with primary expression of Cx47 limited