Characterization of the Human Analogue of a Scrapie-responsive Gene

Dron, Michel; Dandoy-Dron, Françoise; Guillo, Frédéric; Benboudjema, Louisa; Hauw, Jean‐Jacques; Lebon, Pierre; Dormont, Dominique; Tovey, Michaël G.

doi:10.1074/jbc.273.29.18015

Cited by 26 publications

(34 citation statements)

References 11 publications

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“…Scrg1 (for Scrapie responsive gene 1) is a protein encoded by a gene mapped to chromosome 8 in mice and chromosome 4 in humans [29][30][31]. In the neuron, Scrg1 is immunolocalized to dictyosomes …”

Section: Discussionmentioning

confidence: 99%

Autophagy Contributes to Widespread Neuronal Degeneration in Hamsters Infected with the Echigo-1 Strain of Creutzfeldt-Jakob Disease and Mice Infected with the Fujisaki Strain of Gerstmann-Sträussler-Scheinker (GSS) Syndrome

Liberski

Sikorska

Gibson³

et al. 2011

Ultrastructural Pathology

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The authors report here robust autophagy observed by electron microscopy in both the Echigo-1 strain of Creutzfeldt-Jakob disease in hamsters and the Fujisaki strain of Gerstmann-Sträussler-Scheinker disease in mice. In both models, autophagic vacuoles were observed in several cellular compartments. In neuronal cell bodies, autophagic vacuoles of different size were seen. The cytoplasm of some neurons also contained semicircular cisterns equivalent to an early autophasophore. The major target of autophagy was dystrophic neurites, i.e., enlarged neuritic processes--mostly dendrites but also axonal terminals and preterminals. They contained numerous double- or multiple-membrane-bound autophagosomes or autophagolysosomes and large multivesicular bodies. Multivesicular bodies were also observed within autophagic vacuoles, and large multivesicular bodies were seen within synaptic terminals. Some dystrophic neurites was filled almost completely with multivesicular bodies; the latter were occasionally confluent. The authors conclude that autophagy is an important part of neuropathology in prion disease. They also suggest that spongiform vacuoles, a hallmark for the whole group of prion diseases, may in reality originate from autophagic vacuoles.

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Section: Discussionmentioning

confidence: 99%

Autophagy Contributes to Widespread Neuronal Degeneration in Hamsters Infected with the Echigo-1 Strain of Creutzfeldt-Jakob Disease and Mice Infected with the Fujisaki Strain of Gerstmann-Sträussler-Scheinker (GSS) Syndrome

Liberski

Sikorska

Gibson³

et al. 2011

Ultrastructural Pathology

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“…1,2 The Scrg1 gene codes for a 10-kDa protein which contains a N-terminal signal peptide and eight cysteine residues defining an original protein signature. 3,4 The predicted protein is highly conserved in mammals and shows no significant homology with any other known protein. 4,5 Scrg1 is expressed preferentially in the central nervous system and the level of Scrg1 transcripts in primary cultures of neurons is similar to that observed in whole brain indicating that the expression of Scrg1 may be predominant in neurons in vivo.…”

Section: Introductionmentioning

confidence: 99%

SCRG1, a Potential Marker of Autophagy in TSE

Dron¹,

Bailly²,

Béringue³

et al. 2006

Autophagy

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“…TOMM20L, the ligand of TOMM20, which localized at the outer mitochondrial membrane, is associated with metabolism [24]. SCRG1, is associated with neurodegenerative changes observed in transmissible spongiform encephalopathies [25]. SCNM1, sodium channel modifier 1, a zinc finger protein and a putative splice factor, is involved in the splicing of sodium channel transcripts [26].…”

Section: Discussionmentioning

confidence: 99%

Predicting the Likelihood of Postoperative Seizure Status based on mRNA Sequencing in Low-Grade Gliomas

et al. 2017

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Aim:No comprehensive and objective methods yet exist for predicting postoperative seizure. Patients & methods: mRNA-seq data and corresponding postoperative seizure status of 109 low-grade glioma samples were obtained from Chinese Glioma Genome Atlas database and divided into two sets randomly. Logistic regression and receiver operating characteristic analysis with risk score method were used to develop a ten-gene prediction model. Results: Considering gene number and area under the curve of receiver operating characteristic, a ten-gene model was generated which showed an area under the curve of 0.9965 in training set. Patients with high-risk scores had higher probability of postoperative seizure compared with those with low-risk scores. Conclusion: This is the first prediction model for postoperative seizures in gliomas, integrating multiple genes. Clinical application may help patients with postoperative seizure control. Seizure is a common presenting symptom of primary brain tumors. Seizure incidence varies with tumor type, grade and location. Low-grade tumors are more epileptogenic than high-grade tumors [1]. Although most patients (65-90%) with low-grade gliomas (LGGs) experience symptomatic seizures [2], many do not have seizures in spite of similar histology and tumor location. This indicates that the variability of seizure occurrence cannot be explained solely by peritumoral factors or histology, urging us to explore the genetic factors that may be associated with tumor-related seizure. The pathogenesis of tumor-related epilepsy seems to be different from that of idiopathic epilepsy, leading to distinct clinical characteristics and treatment [3].Seizure, as one of the most common postoperative symptom in patients with LGGs [4][5][6][7], when uncontrolled, would badly affect patients' quality of life (QOL), causing cognitive deterioration and may result in significant morbidity [8][9][10][11]. Postoperatively uncontrolled seizures and subsequent use of anti-epileptic drugs (AEDs) would continuously affect QOL and cognitive function [8,10]. Although two-thirds of patients with LGGs would experience seizures after surgery [3], not all LGGs are associated with postoperative seizures, despite of similar histology and tumor location. Therefore, predicting postoperative seizure in patients with diffuse LGGs is valuable [12].Driven by progress in next-generation sequencing, comprehensive interpretation of the tumor biology has been broadly deployed in various cancers [13]. Transcriptome sequencing, especially mRNA-seq, provides a lower cost, more precise insight into the complex transcriptome of the tumors on gene expression, etc. By combining the most advanced technology and the detailed follow-up of patients, we set out to predict the postoperative seizures status, which may help with seizure control postoperatively.

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Characterization of the Human Analogue of a Scrapie-responsive Gene

Cited by 26 publications

References 11 publications

Autophagy Contributes to Widespread Neuronal Degeneration in Hamsters Infected with the Echigo-1 Strain of Creutzfeldt-Jakob Disease and Mice Infected with the Fujisaki Strain of Gerstmann-Sträussler-Scheinker (GSS) Syndrome

Autophagy Contributes to Widespread Neuronal Degeneration in Hamsters Infected with the Echigo-1 Strain of Creutzfeldt-Jakob Disease and Mice Infected with the Fujisaki Strain of Gerstmann-Sträussler-Scheinker (GSS) Syndrome

SCRG1, a Potential Marker of Autophagy in TSE

Predicting the Likelihood of Postoperative Seizure Status based on mRNA Sequencing in Low-Grade Gliomas

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