ライフサイエンスコーパス: lower motor neuron

1 sease characterized by the loss of upper and lower motor neurons.

2 terized by the progressive loss of upper and lower motor neurons.

3 (ALS) is a fatal disease affecting upper and lower motor neurons.

4 aracterized by progressive loss of upper and lower motor neurons.

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

6 of injuries affecting the spinal cord or the lower motor neurons.

7 length-dependent axonal degeneration of the lower motor neurons.

8 roup of pathologies that affect upper and/or lower motor neurons.

9 acterized by early onset and degeneration of lower motor neurons.

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

11 by muscular atrophy due to loss of upper and lower motor neurons.

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

13 recessive disorder characterized by loss of lower motor neurons.

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

15 axonopathies affecting peripheral nerves and lower motor neurons.

16 ong CNS pathway comprising corticospinal and lower motor neurons.

17 ve disease that mainly affects the upper and lower motor neurons.

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

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

20 euronal synapses for restoring activation of lower motor neurons after stroke.

21 n = 3 SOD1 I113T mutation carriers, diffuse lower motor neuron and upper motor neuron signs evolved

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

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

24 Both models exhibit loss of lower motor neurons and neurons of the deep cerebellar n

25 to the progressive degeneration of upper and lower motor neurons and of neocortical areas, respective

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

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

28 nction (NMJ) is the unique interface between lower motor neurons and skeletal muscle fibers and is in

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

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

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

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

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

34 It is not known why lower motor neurons are particularly sensitive to the lo

35 In the central nervous system, lower motor neurons are selectively affected, whereas pa

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

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

38 observable prodrome invariably involved the lower motor neuron axis.

39 different genes that lead to degeneration of lower motor neuron axons.

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

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

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

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

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

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

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

47 ceptor (AR) gene, leading to AR aggregation, lower motor neuron death, and muscle atrophy.

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

49 akness, with signs and symptoms of upper and lower motor neuron degeneration in multiple myotomes, wi

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

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

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

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

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

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

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

57 of corticospinal tract degeneration without lower motor neuron degeneration.

58 ive cytoplasmic inclusions and predominantly lower motor neuron degeneration.

59 a neurodegenerative disease characterized by lower motor neuron degeneration.

60 ited neuromuscular disorder characterized by lower motor neuron degeneration.

61 sive and heterogeneous group of upper and/or lower motor neuron degenerative disorders, in which the

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

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

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

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

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

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

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

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

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

71 of neuromuscular disorders characterized by lower motor neuron dysfunction, slowly progressive lengt

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

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

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

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

76 Stack redress the imbalance by presenting a lower motor neuron homunculus.

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

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

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

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

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

82 ent plasticity of synapses between upper and lower motor neurons in the spinal cord, downstream of su

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

84 We also observe selective death of lower motor neurons in vivo with worsening motor behavio

85 2-null animals, we observe smaller upper and lower motor neurons in vivo.

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

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

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

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

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

91 new technologies sensitive to both upper and lower motor neuron involvement may ultimately resolve co

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

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

94 nversion, namely NfL levels, depression, and lower motor neuron involvement, differ from those drivin

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

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

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

98 m cell (iPSC)-induced excitatory neurons and lower motor neurons, iPSC-derived hippocampal dentate gy

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

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

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

102 adily established, functional assessments of lower motor neuron (LMN) innervation of forelimb muscles

103 aired body region attribute to impairment of lower motor neuron (LMN) or UMN or mixed LMN and UMN?

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

105 Lower motor neuron (LMN) syndromes typically present wit

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

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

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

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

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

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

112 sease characterized by the loss of upper and lower motor neurons (MNs).

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

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

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

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

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

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

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

120 ease progression, and in ALS patient-derived lower motor neurons, our AAV vector transduced >95% of c

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

122 o of the individuals, both showing upper and lower motor neuron pathology.

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

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

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

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

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

128 for definite, probable, or possible ALS with lower motor neuron signs by modified El Escorial Criteri

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

130 related to the balance of clinical upper and lower motor neuron signs, upper versus lower limb onset

131 56, 15, and 12 months), and 2 remain without lower motor neuron signs.

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

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

134 The lower motor neuron syndrome after irradiation to the spi

135 197G>T p.G66V in CHCHD10 is the cause of the lower motor neuron syndrome LOSMoN/SMAJ.

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

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

138 utoimmune pathogenesis of the paraneoplastic lower motor neuron syndrome.

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

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

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

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

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

144 clerosis (ALS) is a disease of the upper and lower motor neurons, to date none of the compounds in cl

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

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

147 a neurodegenerative disease of the upper and lower motor neurons with varying ages of onset, progress

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

149 almost exclusively as a disease of upper and lower motor neurons, with muscle changes interpreted as

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

151 aracterized by the degeneration of upper and lower motor neurons within the brain and spinal cord.