HUGO ID Detailed Result 14374


HUGO ID 14374
Symbol NLRP1
Name NLR family, pyrin domain containing 1
#Occurrence 76
#Paper 2

 


PMID Match String Actual String Score Flanking text Edited by Edit
8922414NACNAC0.3the effects of agents such as N -acetyl-L -cysteine (NAC), NAC which reduce free radical damage 
8922414NACNAC0.3mice were given a 1% solution of the glutathione precursor NAC in their drinking water for a period of 9 weeks 
8922414NACNAC0.3examination of these animals revealed that wobbler mice treated with NAC exhibited (1) 1 a significant reduction in motor neuron loss 
8922414NACNAC0.3neurons in wobbler mice and demonstrate that oral administration of NAC effectively reduces the degree of motor degeneration in wobbler mice 
8922414NACNAC0.3The compound N -acetyl-L -cysteine (NAC) NAC has been shown to inhibit ROS thus increasing the viability 
8922414NACNAC0.3In vivo NAC also has been shown to reduce the toxicity of compounds 
8922414NACNAC0.3In humans extensive clinical experience with NAC suggests that it is a compound of low toxicity most 
8922414NACNAC0.3NAC seems to exert these effects by acting directly as an 
8922414NACNAC0.3To examine the effects of NAC on motor neuron degeneration in vivo we treated wobbler mice 
8922414NACNAC0.3degeneration in vivo we treated wobbler mice per os with NAC over a period of 9 weeks 
8922414NACNAC0.3The results demonstrate that NAC treatment reduces the decline in several important morphological and functional 
8922414NACNAC-treated0.3solutions were adjusted to the same pH and molarity as NAC-treated animals (61.2 61.2 m M and served as control agents 
8922414NACNAC-treated0.3Four-week-old NAC-treated mice drank an average of 3.4 _amp_#177 0.6 ml per 
8922414NACNAC0.3The mean quantity of NAC ingested was ~2.3 mg/gm mg gm body weight 
8922414NACNAC0.3The mean quantity of NAC received was 2.1 mg/gm mg gm body weight 
8922414NACNAC-treated0.3Even within the NAC-treated group the distinction between wr/wr wr wr and NW mice 
8922414NACNAC-treated0.3of the facial nerve for wr/wr wr wr NW and NAC-treated wr/wr wr wr and NW mice is shown in Figure 
8922414NACNAC-treated0.3In NAC-treated wr/wr wr wr mice [wr/wr(N)] wr wr N this reduction 
8922414NACNAC0.3nerve as compared with NW controls wobbler animals treated with NAC do not show this loss of motor axons instead giving 
8922414NACNAC-treated0.3the distribution of axon areas in wr/wr wr wr and NAC-treated wr/wr wr wr mice respectively 
8922414NACNAC-treated0.3In contrast NAC-treated wobbler mice do not show a marked reduction in axon 
8922414NACNAC0.3the wobbler controls are significantly different from both the wr NAC treatment and NW groups pair-wise comparisons wr/wr wr wr vs 
8922414NACNAC0.3given above demonstrate that oral treatment of wobbler mice with NAC can reduce significantly the degree of axonal atrophy and loss 
8922414NACNAC0.3To examine the effects of NAC on neuromuscular aspects of the forelimb we took 10 micro 
8922414NACNAC0.3neurons by 9 weeks of age wobbler mice treated with NAC exhibited significantly greater numbers of ChAT-positive neurons than wr/wr wr 
8922414NACNAC-wobbler0.3However NAC-wobbler mice still exhibited a substantial loss of ChAT-positive neurons as 
8922414NACNAC0.3To determine the effects of NAC treatment on the innervation targets of these spinal motor neurons 
8922414NACNAC0.3As shown in Figure 4 A B animals treated with NAC show a significant increase in the overall mass of the 
8922414NACNAC0.3Thus treatment with NAC reduces the degree of overt muscular atrophy that is induced 
8922414NACNAC0.3To delineate more clearly the effects of NAC on muscle morphology within the distal forelimb we determined cross-sectional 
8922414NACNAC0.3shown in Figure 4 C indicate that animals treated with NAC show a marked increase in mean fiber area (~603 ~603 
8922414NACNAC0.3These data demonstrate that oral administration of NAC can reduce significantly motor neuron loss and axonal atrophy in 
8922414NACNAC0.3These results demonstrate that although wobbler mice receiving NAC do exhibit a significant reduction in forelimb function they perform 
8922414NACNAC0.3These data suggest that treatment with NAC does result in some reduction in the functional losses that 
8922414NACNAC0.3Previous work has suggested that NAC acts in part by increasing intracellular supplies of cysteine the 
8922414NACNAC0.3To determine the ability of NAC to enhance the free-radical scavenging ability of the GPx system 
8922414NACNAC0.3As shown in Figure 6 animals that received NAC in their drinking water exhibited a substantial increase in GPx 
8922414NACNAC0.3similar to that of NW mice that did not receive NAC supplementation 
8922414NACNAC0.3To further assess the ability of NAC to effect GPx activity levels in non-wobbler mice we injected 
8922414NACNAC0.3(glucose glucose treatment group daily with 1 mg/gm mg gm NAC for 3 d before the analysis 
8922414NACNAC0.3These results demonstrate the ability of NAC to enhance antioxidant activity in tissues affected by the wobbler 
8922414NACNAC0.3The results demonstrate that daily oral administration of NAC significantly reduces the degeneration of lower motor neurons in wobbler 
8922414NACNAC0.3For motor axons of the facial nerve application of NAC resulted in a generalized increase in axon number and caliber 
8922414NACNAC0.3Within the cervical spinal cord NAC reduced losses of choline acetyltransferase-positive neurons 
8922414NACNAC0.3NAC has been shown previously in several of these paradigms to 
8922414NACNAC0.3The ability of NAC to reduce the loss of these lower motor neurons in 
8922414NACNAC0.3in the cervical spinal cord suggests a means by which NAC may act to reduce local ROS levels 
8922414NACNAC0.3Previous work also demonstrates that NAC directly supports glutathione synthesis in neural cells thus reducing intracellular 
8922414NACNAC0.3These data suggest that NAC may exert survival-promoting effects in a manner independent of its 
8922414NACNAC0.3NAC has been used previously in a limited trial over a 
8922414NACNAC0.3no net clinical improvement was observed in patients treated with NAC segregation of ALS cases into bulbar and limb onset groups 
8922414NACNAC0.3In ALS cases of bulbar onset NAC did not improve survival 
8922414NACNAC0.3However in patients with the limb onset form NAC does seem to improve survival (NAC NAC 74% 28/38; 28 
8922414NACNAC0.3limb onset form NAC does seem to improve survival (NAC NAC 74% 28/38; 28 38 vehicle 51% 22/43) 22 43 
8922414NACNAC0.3It is interesting to speculate that the differential availability of NAC to these two neural populations may underlie the differences in 
8922414NACNAC0.3function over a similar time frame than those treated with NAC 
8922414NACNAC0.3For example to be accessible to motor neurons NAC must be taken up via contact with somatic tissue through 
8922414NACNAC0.3Oral administration of NAC does result in enhanced GPx activity in the cervical spinal 
8922414NACNAC0.3axonal transport may be impaired in their ability to accumulate NAC and its metabolites thus limiting its potential therapeutic effects 
8922414NACNAC0.3In addition it is important to realize that NAC may be affecting only a portion of the pathways involved 
8922414NACNAC0.3Despite this the use of agents such as NAC would seem to be a practical approach to enhancing cell 
8922414NACNAC0.3intrathecal implantation of proteins which are quite labile in vivo NAC can be given per os over dispersed intervals 
8922414NACNAC0.3NAC is also an inexpensive drug possessing few side effects and 
8922414NACNAC0.3to that of several human neurodegenerative disorders the application of NAC may prove useful in treating other mammalian neurodegenerative diseases 
8922414NACNAC0.3NAC reduces axonal loss in wobbler facial nerve 
8922414NACNAC-treated0.3Control-treated wr/wr wr wr mice n = 17 NAC-treated wr/wr wr wr mice n = 11 NW mice n 
8922414NACNAC0.3NAC prevents losses in axon caliber in wobbler mice 
8922414NACNAC-treated0.3C Axon distribution of NAC-treated wr/wr wr wr mice n = 6 
8922414NACNAC0.3NAC reduces the loss of ChAT-positive neurons in the cervical spinal 
8922414NACNAC0.3NAC reduces the atrophy of forelimb muscles in wobbler mice 
8922414NACNAC0.3NAC reduces functional losses in the forelimb of wobbler mice 
8922414NACNAC0.3NAC treatment augments glutathione peroxidase (GPx) GPx levels in the cervical 
12663085NACNAC0.3of MG and 3-DG was attenuated by N -acetylcysteine (NAC) NAC 
12663085NACNAC0.3NAC can raise intracellular GSH levels and thereby provide cells with 
12663085NACNAC0.3In addition NAC also reacts with MG directly and reversibly to form the 
12663085NACNAC0.3To test this possibility we added glutathione-augmenting agents such as NAC and glutathione ethyl ester (GEE) GEE 24 h before MG