ライフサイエンスコーパス: lower motor neuron

1 w levels of SMN protein, primarily affecting lower motor neurons.

2 uscular disease characterized by the loss of lower motor neurons.

3 rophy (SBMA) that leads to selective loss of lower motor neurons.

4 recessive disorder characterized by loss of lower motor neurons.

5 disease caused by degeneration of upper and lower motor neurons.

6 axonopathies affecting peripheral nerves and lower motor neurons.

7 ong CNS pathway comprising corticospinal and lower motor neurons.

8 or muscle in non-cell autonomous toxicity of lower motor neurons.

9 hic lateral sclerosis when there is a marked lower motor neuron abnormality.

10 characterized by the death of both upper and lower motor neurons and by 3- to 5-yr median survival po

11 and result in the observed specific loss of lower motor neurons and muscle wasting.

12 his neuromuscular disorder which affects the lower motor neurons and proximal muscles of the limbs an

13 f mutant human SOD1 mRNA levels in upper and lower motor neurons and significant improvements in mult

14 (SMN1) causes the selective degeneration of lower motor neurons and subsequent atrophy of proximal s

15 , SMA pathology was thought to be limited to lower motor neurons and the skeletal muscles they contro

16 ce of any detectable degenerative changes in lower motor neurons and with a molecular profile distinc

17 en in the neuromuscular junctions (NMJs) and lower motor neurons, and selective reduction of toxic mu

18 ve spinal muscular atrophy has revealed that lower motor neurons appear to have a specific vulnerabil

19 ly progressive neurodegenerative pathways in lower motor neurons around the time of disease onset rat

20 identified a defect in repetitive firing of lower motor neurons as a novel contributor to intensive

21 uitously expressed gene (SMN1), with loss of lower motor neurons being the primary pathological signa

22 ive disease that primarily affects upper and lower motor neurons, but also frontotemporal and other r

23 ed SMN protein result in the degeneration of lower motor neurons, but it remains unclear whether othe

24 Kennedy disease, a degenerative disorder of lower motor neurons caused by a CAG/glutamine expansion

25 s and post-synaptic motor endplates, loss of lower motor neuron cell bodies and denervation of neurom

26 ting neurological disease marked by isolated lower motor neuron death and subsequent atrophy of skele

27 enerative disease characterized by upper and lower motor neuron death with ascending paralysis leadin

28 ease and all cases showed combined upper and lower motor neuron degeneration (amyotrophic lateral scl

29 gical analyses indicated a similar extent of lower motor neuron degeneration in SODMutM maintained on

30 tion of needle electromyographic evidence of lower motor neuron degeneration into diagnostic criteria

31 was degeneration of the motor cortex without lower motor neuron degeneration or involvement of other

32 cterized as exhibiting adult-onset upper and lower motor neuron degeneration, but closer examination

33 ult-onset disease characterized by upper and lower motor neuron degeneration, muscle wasting and para

34 that is characterized by selective upper and lower motor neuron degeneration, the pathogenesis of whi

35 acterized by adult-onset muscle weakness and lower motor neuron degeneration.

36 of corticospinal tract degeneration without lower motor neuron degeneration.

37 ive cytoplasmic inclusions and predominantly lower motor neuron degeneration.

38 a neurodegenerative disease characterized by lower motor neuron degeneration.

39 ited neuromuscular disorder characterized by lower motor neuron degeneration.

40 Although isolated lower motor neuron disease has been reported as a parane

41 A) is a fully penetrant, autosomal recessive lower motor neuron disease in domestic cats that clinica

42 Here we show linkage of a lower motor neuron disease to a region of 4 Mb at chromo

43 any forms of spinal muscular atrophy, a pure lower motor neuron disease, have been characterized clin

44 loss-of-function of HSJ1 is linked to a pure lower motor neuron disease, strongly suggesting that HSJ

45 diseases: amyotrophic lateral sclerosis and lower motor neuron disease.

46 nd mutations in its gene are associated with lower motor neuron disease.

47 Only 17% of women with complete lower motor neuron dysfunction affecting the S2-S5 spina

48 clerosis (ALS) is characterized by upper and lower motor neuron dysfunction and loss, rapidly progres

49 cranial neuropathy causing acute unilateral lower motor neuron facial paralysis.

50 In all cohort members, lower motor neuron features were static or only slowly p

51 or pathological hallmark of SMA is a loss of lower motor neurons from spinal cord and peripheral nerv

52 to neurodegenerative disorders of upper and lower motor neurons, hereditary spastic paraplegia (HSP)

53 rovides an index of the number of functional lower motor neurons in a given muscle.

54 ite the vulnerability of these spinal axons, lower motor neurons in ALS2-deficient mice were preserve

55 disease (PD), the degeneration of upper and lower motor neurons in amyotrophic lateral sclerosis (AL

56 sease characterized by the loss of upper and lower motor neurons in the brain and spinal cord.

57 e characterized by degeneration of upper and lower motor neurons in the brain and spinal cord.

58 essive disorder characterized by the loss of lower motor neurons in the spinal cord.

59 rtex, and spinal/bulbar motor neurons (SMN; "lower motor neurons") in spinal cord and brainstem.

60 utput and selectively degenerate (along with lower motor neurons) in amyotrophic lateral sclerosis.

61 Selective death of lower motor neurons, including those innervating the lim

62 disrupt prenatal developmental processes in lower motor neurons, influencing neuronal outgrowth, axo

63 All 6 exhibited severe lower motor neuron involvement in addition to cognitive

64 phenotype with a mixed picture of upper and lower motor neuron involvement reminiscent of amyotrophi

65 l sclerosis that acknowledged both upper and lower motor neuron involvement was attributed to Jean-Ma

66 equiring dinical evidence for both upper and lower motor neuron involvement, should be modified; ie,

67 physiological, or histopathological signs of lower motor neuron involvement.

68 eurodegenerative disease with both upper and lower motor neuron involvement.

69 lity and is characterized by degeneration of lower motor neurons leading to muscle wasting.

70 aracterized by the selective degeneration of lower motor neurons, leading to muscle atrophy and, in t

71 A type of lower motor neuron (LMN) disease inherited as autosomal

72 All subjects had lower motor neuron (LMN) signs and electrophysiological

73 Lower motor neuron (LMN) syndromes typically present wit

74 d white matter tract damage in patients with lower motor neuron (LMN)-predominant disease compared wi

75 Loss of lower motor neurons (LMNs) and large-caliber axons was c

76 and/or progressive degeneration of upper and lower motor neurons (LMNs) causes neurological symptoms

77 SMA is characterized by alpha-lower motor neuron loss and muscle atrophy, however, the

78 able electrophysiological biomarker to track lower motor neuron loss in ALS.

79 Severe SMA causes lower motor neuron loss, impaired myofiber development,

80 urodegenerative disorder affecting upper and lower motor neurons (MNs).

81 aracterized by the degeneration of upper and lower motor neurons (MNs).

82 Spinal muscular atrophy is a disorder of lower motor neurons, most commonly caused by recessive m

83 plex and not related simply to the degree of lower motor neuron muscle wasting but, rather, depend on

84 Mutant TDP-43-dependent degeneration of lower motor neurons occurs without: (i) loss of TDP-43 f

85 s a common disorder characterized by loss of lower motor neurones of the spinal cord.

86 ilhier) had already recognized a progressive lower motor neuron-only syndrome within a broader, clini

87 Motor function and upper and lower motor neuron pathology were examined in ALS2-defic

88 tive disorder in which the loss of upper and lower motor neurons produces progressive weakness and ev

89 Patients have selective loss of lower motor neurons resulting in muscle weakness, paraly

90 s characterized by degeneration of upper and lower motor neurons, resulting in progressive weakness a

91 We show here that dysmyelinated lower motor neurons retract and regenerate dysfunctional

92 amyotrophic lateral sclerosis by its lack of lower motor neuron signs and long survival.

93 Low NFL levels in patients with lower motor neuron signs might be a prognostic indicator

94 56, 15, and 12 months), and 2 remain without lower motor neuron signs.

95 cend, causing flaccid tetraparesis and other lower motor neuron signs.

96 disease characterized by combined upper and lower motor neuron symptoms and signs with onset prior t

97 The lower motor neuron syndrome after irradiation to the spi

98 197G>T p.G66V in CHCHD10 is the cause of the lower motor neuron syndrome LOSMoN/SMAJ.

99 odal antigens as novel autoimmune targets in lower motor neuron syndrome provide new insights into th

100 woman with breast cancer and paraneoplastic lower motor neuron syndrome whose serum contained autoan

101 utoimmune pathogenesis of the paraneoplastic lower motor neuron syndrome.

102 in the development of the extraocular muscle lower motor neuron system.

103 A)) receptors in their CNS, particularly the lower motor neurons, than do wild-type mice, indicating

104 to rescue or replace degenerating upper and lower motor neurons through endogenous recruitment or au

105 y is a key factor regulating the response of lower motor neurons to injury.

106 ansmitter that relays neural excitation from lower motor neurons to muscles.

107 Understanding the molecular basis of lower motor neuron vulnerability in disorders such as sp

108 ated neurofilaments (NP-NF) in the upper and lower motor neurons was investigated in the rat, the com

109 aracterized by progressive loss of upper and lower motor neurons, with a median survival of 2-3 years

110 l process that preferentially affects lumbar lower motor neurons, with or without additional upper mo