Insight of vitellogenesis patterns: A comparative analysis of the differences between the primary and secondary vitellogenesis period in the ovary, hepatopancreas, and muscle of mud crab, scylla paramamosain.

The mud crab, Scylla paramamosain, has abundant nutrients in its edible parts, ovary, hepatopancreas, and muscle during the ovarian maturation stage. The ovary of S. paramamosain can re-mature after spawning during the secondary ovarian maturation period. We aimed to analyze the characteristics of the first vitellogenesis period (FVP) and second vitellogenesis period (SVP) of S. paramamosain during ovarian maturation to understand the differences in vitellogenesis patterns between the first and second ovarian maturation periods. Accordingly, the gonadosomatic index (GSI) and hepatopancreatic index (HSI), the external and histological characteristics of the ovary and hepatopancreas, the Sp-Vg (vitellogenin, Vg) expression levels in the hepatopancreas and ovary, and the dynamics of the biochemical components in the ovary, hepatopancreas, and muscle were determined. Based on the results, the GSI was significantly positively correlated with HSI during the FVP and significantly negatively correlated with HSI from stage Ⅳ to stage Ⅴ of the SVP. A significant difference was found between the FVP and SVP in the hepatopancreas. Notably, the hepatopancreas displayed a gradual degeneration trend during the SVP. The expression level of Sp-Vg was significantly higher in the hepatopancreas than that in the ovary during the FVP and SVP. Seventeen amino acids were detected in the hepatopancreas, ovary, and muscle during the FVP and SVP, with glutamate as the predominant amino acid. During the FVP and SVP, the C16:0 and C18:1n9c were the dominant fatty acids in the hepatopancreas and ovary, the MUFA gradually increased in the ovary and hepatopancreas, and a significant difference was found in the dynamic trend of the HUFA and SFA contents from stage Ⅳ to stage Ⅴ between the FVP and SVP. These findings indicate that the ovary can re-mature after spawning in S. paramamosain and can maintain the status of the first ovarian maturation; however, the hepatopancreas gradually degenerate during the SVP.

Autism-Risk Gene necab2 Regulates Psychomotor and Social Behavior as a Neuronal Modulator of mGluR1 Signaling.

Background: De novo deletion of the neuronal calcium-binding protein 2 (NECAB2) locus is associated with idiopathic autism spectrum disorders (ASDs). The in vivo function of NECAB2 in the brain remains largely elusive. Methods: We investigated the morphological and behavioral profiles of both necab2 knock-out and overexpression zebrafish models. The expression pattern and molecular role of necab2 were probed through a combination of in vitro and in vivo assays. Results: We show that Necab2 is a neuronal specific, cytoplasmic, and membrane-associated protein, abundantly expressed in the telencephalon, habenula, and cerebellum. Necab2 is distributed peri-synaptically in subsets of glutamatergic and GABAergic neurons. CRISPR/Cas9-generated necab2 knock-out zebrafish display normal morphology but exhibit a decrease in locomotor activity and thigmotaxis with impaired social interaction only in males. Conversely, necab2 overexpression yields behavioral phenotypes opposite to the loss-of-function. Proteomic profiling uncovers a role of Necab2 in modulating signal transduction of G-protein coupled receptors. Specifically, co-immunoprecipitation, immunofluorescence, and confocal live-cell imaging suggest a complex containing NECAB2 and the metabotropic glutamate receptor 1 (mGluR1). In vivo measurement of phosphatidylinositol 4,5-bisphosphate further substantiates that Necab2 promotes mGluR1 signaling. Conclusions: Necab2 regulates psychomotor and social behavior via modulating a signaling cascade downstream of mGluR1.

Genetic diversity and population structure analysis of Lateolabrax maculatus from Chinese coastal waters using polymorphic microsatellite markers.

In order to provide valuable guidelines for the conservation of germplasm of Lateolabrax maculatus, the genetic diversity and population structure analysis were evaluated for eight geographic populations along coastal regions of China, using 11 microsatellite DNA markers. The genetic parameters obtained showed that, eight populations can be clustered into two groups, the Northern group and the Southern group, concordant with their geographical positions. The UPGMA tree constructed according to the Nei's genetic distance along with the structure analysis and discriminant analysis of principal component also supported this result. This might be explained by the geographic separation and the divergent environmental conditions among the populations. It's worth noting that, QD (Qingdao) population from northern area was assigned to the Southern group and showed a close genetic relationship and similar genetic constitution with the southern populations. We speculated that large scales of anthropogenic transportation of wild fries from QD populations to the southern aquaculture areas in history should be the primary cause. The populations from GY (Ganyu), RD (Rudong) and BH (Binhai) had higher genetic diversity and showed limited genetic exchange with other populations, indicating better conservation of the natural resources in these regions. All populations were indicated to have experienced bottleneck events in history.

An optimized procedure for detection of genetically modified DNA in refined vegetable oils.

In this study, the amplifiable DNA from refined vegetable oils was isolated by using commercial DNA extraction kits based on the CTAB method in combination with nucleic acid enrichment, and then the presence of genetically modified (GM) soybean and maize DNA in the oils was traced by PCR. The results showed that the duration and intensity of heating had no significant effect on the DNA stability and concentration in oils for a short period, suggesting that DNA in oils could be stably reserved for a certain time, thus making it possible to trace down refined vegetable oils reliably and effectively. The results provided a set of primers suitable for systematic GM oil detection. More importantly, this study made an important contribution to the economical and reliable detection of GM vegetable oils regarding food authenticity issues.

A chromosome-level genome of the mud crab (Scylla paramamosain estampador) provides insights into the evolution of chemical and light perception in this crustacean.

Mud crabs, found throughout the Indo-Pacific region, are coastal species that are important fisheries resources in many tropical and subtropical Asian countries. Here, we present a chromosome-level genome assembly of a mud crab (Scylla paramamosain). The genome is 1.55 Gb (contig N50 191 kb) in length and encodes 17,821 proteins. The heterozygosity of the assembled genome was estimated to be 0.47%. Effective population size analysis suggested that an initial large population size of this species was maintained until 200 thousand years ago. The contraction of cuticle protein and opsin genes compared with Litopenaeus vannamei is assumed to be correlated with shell hardness and light perception ability, respectively. Furthermore, the analysis of three chemoreceptor gene families, the odorant receptor (OR), gustatory receptor (GR) and ionotropic receptor (IR) families, suggested that the mud crab has no OR genes and shows a contraction of GR genes and expansion of IR genes. The numbers of the three gene families were similar to those in three other decapods but different from those in two nondecapods and insects. In addition, IRs were more diversified in decapods than in nondecapod crustaceans, and most of the expanded IRs in the mud crab genome were clustered with the antennal IR clades. These findings suggested that IRs might exhibit more diverse functions in decapods than in nondecapods, which may compensate for the smaller number of GR genes. Decoding the S. paramamosain genome not only provides insight into the genetic changes underpinning ecological traits but also provides valuable information for improving the breeding and aquaculture of this species.

The complete mitochondrial genome sequence and gene organization of Cryodraco antarcticus (Perciformes, Channichthyidae) with phylogenetic consideration.

The complete mitochondrial genome DNA sequence of Cryodraco antarcticus was 17,857 bp in size. It consists of 13 protein-coding genes, 2 ribosomal RNAs, 22 transfer RNAs, and one control region. Among 22 tRNA genes, 8 tRNAs were encoded on the L-strand. The overall base composition of the genome is 26.45% for A, 25.96% for T, 29.78% for C, and 17.81% for G. The phylogenetic tree suggested C. antarcticus was genetically closest to some species in family Channichthyidae. This study could provide valuable information for further studies on population structure, conservation genetics and molecular evolution of C. antarcticus.

Genome survey, high-resolution genetic linkage map construction, growth-related quantitative trait locus (QTL) identification and gene location in Scylla paramamosain.

Scylla paramamosain is one of the most economically important crabs in China. In this study, the first genome survey sequencing of this crab was performed, and the results revealed that the estimated genome size was 1.21 Gb with high heterozygosity (1.3%). Then, RAD technology was used to construct a high-resolution linkage map for this species. A total of 24,444 single nucleotide polymorphism (SNP) makers were grouped into 47 linkage groups. The total length of the linkage groups was 3087.53 cM with a markers interval of 0.92 cM. With the aid of transcriptome and genome scaffold data, 4,271 markers were linked to genes, including several important growth-related genes such as transforming growth factor-beta regulator I, immune related-gene C-type lectin and ecdysone pathway gene broad-complex-like protein. Further, 442 markers, representing 279 QTLs, associated with 24 traits were identified, and of these markers, 78 were linked to genes. Some interesting genes, such as dedicator of cytokinesis protein 3, tenascin-X and DNA helicase MCM8, were believed to have important relationship with specific traits and merit further exploration. The results of this study will accelerate the genetic improvement and genome sequencing analysis of the mud crab.

Tissue-based transcriptomics of Chionodraco hamatus: Sequencing, de novo assembly, annotation and marker discovery.

Here, we explored the liver, heart and muscle tissue transcriptome of the haemoglobinless Antarctic fish Chionodraco hamatus using the Illumina paired-end RNA sequencing. A total of 114,028 unigenes with a mean length of 794.24 bp was produced. Annotation of these unigenes showed that 29.16% and 35.52% of them had hits in the nucleotide (Nt) and protein (Nr) databases, respectively. In addition, 29.10% and 35.28% unigenes were annotated in the SwissProt and TrEMBL databases while 23.27% and 21.08% of unigenes were annotated in the conserved domain (CCD) and protein family (PFAM) databases, respectively. The results of eukaryotic orthologous groups (KOG) classification analysis showed that around 21.36% of unigenes could be mapped. Differential gene expression analysis indicated that 16,331, 16,291 and 13,262 differentially expressed genes (DEG) could be screened between muscle and heart, muscle and liver and heart and liver, respectively. A significant enrichment analysis of these DEGs revealed their implication in important biological processes, molecular functions, cellular components and diverse pathways. Furthermore, a total of 24,455 simple sequence repeats (SSR) were detected from the generated tissue transcriptome. The transcriptome data produced in this study will constitute an important resource for improving our knowledge of C. hamatus functional genomics and will facilitate future studies regarding this species.

An amine oxidase gene from mud crab, Scylla paramamosain, regulates the neurotransmitters serotonin and dopamine in vitro.

Amine oxidase, which participates in the metabolic processing of biogenic amines, is widely found in organisms, including higher organisms and various microorganisms. In this study, the full-length cDNA of a novel amine oxidase gene was cloned from the mud crab, Scylla paramamosain, and termed SpAMO. The cDNA sequence was 2,599 bp in length, including an open reading frame of 1,521 bp encoding 506 amino acids. Two amino acid sequence motifs, a flavin adenine dinucleotide-binding domain and a flavin-containing amine oxidoreductase, were highly conserved in SpAMO. A quantitative real-time polymerase chain reaction analysis showed that the expression level of SpAMO after quercetin treatment was time- and concentration-dependent. The expression of SpAMO tended to decrease and then increase in the brain and haemolymph after treatment with 5 mg/kg/d quercetin; after treatment with 50 mg/kg/d quercetin, the expression of SpAMO declined rapidly and remained low in the brain and haemolymph. These results indicated that quercetin could inhibit the transcription of SpAMO, and the high dose (50 mg/kg/d) had a relatively significant inhibitory effect. SpAMO showed the highest catalytic activity on serotonin, followed by dopamine, ß-phenylethylamine, and spermine, suggesting that the specific substrates of SpAMO are serotonin and dopamine. A bioinformatics analysis of SpAMO showed that it has molecular characteristics of spermine oxidase, but a quercetin test and enzyme activity study indicated that it also functions like monoamine oxidase. It is speculated that SpAMO might be a novel amine oxidase in S. paramamosain that has the functions of both spermine oxidase and monoamine oxidase.

The complete mitochondrial genome of Electrona carlsbergi (Myctophiformes, Myctophidae) with phylogenetic consideration.

The complete mitochondrial genome of Electrona carlsbergi was obtained, which was 18,282 bp in size and including 13 protein-coding genes, 2 ribosomal RNAs, 23 transfer RNAs and 1 control region. The overall nucleotide composition is 27.92% for A, 24.66% for T, 30.90% for C and 16.52% for G. Among 23 tRNA genes, 8 tRNAs were encoded on the L-strand. Further, the phylogenetic tree, which based on complete mtDNA sequences, revealed that the E. carlsbergi was genetically closest to species E. antarctica and Krefftichthys anderssoni. This study could provide a basic data for the studies on evolution for low temperature adaptability, stock evaluation and conservation genetics.

The complete mitochondrial genome of Chionodraco rastrospinosus (Notothenioidei: Channichthyidae) with phylogenetic consideration.

In this study, the complete mitochondrial genome of Chionodraco rastrospinosus was obtained, which was 17598 bp including 2 ribosomal RNAs, 13 protein-coding genes, 22 transfer RNAs, and a non-coding control region. The length of D-loop was 1332 bp and its contents of A, T, C, and G were 30.3%, 27.6%, 26.8%, and 15.3%. The complete mtDNA sequences of C. rastrospinosus and other 14 species were used to reconstruct the phylogenetic tree, suggested that C. rastrospinosus was closest to two species of Chionodraco. The study would provide a basic data for further research on population structure, conservation genetics and molecular evolution of C. rastrospinosus.

The complete mitochondrial genome of Neopagetopsis ionah (Channichthyidae, neopagetopsis) with phylogenetic consideration.

In this study, the complete mitochondrial genome of Neopagetopsis ionah was obtained, which was 17,634 bp including two ribosomal RNAs, 13 protein-coding genes, 22 transfer RNAs, and a non-coding control region. In 13 protein-coding genes, there types of initiation codon (ATC, ATG, and GTG) and four types of stop codons (TAA, TAG, TA, and T) were identified. Among the 22 transfer RNAs, eight tRNAs were encoded by L-strand. The length of D-loop was 1519 bp and its contents of A, T, C, and G were 26.9%, 27.6%, 17.5%, and 30%, respectively. The complete mtDNA sequences of N. ionah and other 13 species were used to reconstruct the phylogenetic tree suggested that N. ionah was closest to some species of Channichthyidae. The study would provide a basic data for further research on population structure, conservation genetics and molecular evolution of N. ionah.

Isolation and characterization of the mitochondrial genome of Gymnodraco acuticeps (Perciformes: Bathydraconidae) with phylogenetic consideration.

Gymnodraco acuticepsis is an Antarctic fish living in the Southern Ocean. Until now, studies on G. acuticeps are still limited. As an Antarctic fish, obtaining and characterization of the mitochondrial genome of G. acuticeps will be important for elucidation of the mechanism of cold-adapting evolution in mitochondrion. In this study, we first isolated and characterized the mitochondrial genome sequence of G. acuticeps with 15,987 bp in length. It contained of 34 genes (12 protein-coding genes, 20 transfer RNA genes, 2 ribosomal RNA genes) and a partial putative control region. Gene organization and nucleotide composition of obtained mito-genome were similar to those of other Antarctic fish. Twenty-eight genes were encoded by heavy strand, while six genes were encoded by light strand. Further, the phylogenetic tree, which based on 12 protein-coding genes, revealed that the G. acuticeps was genetically closest to species Parachaenichthys charcoti among 18 species. We hope this work would be helpful for the population genetics and molecular evolution studies.

The Gut Microbial Community of Antarctic Fish Detected by 16S rRNA Gene Sequence Analysis.

Intestinal bacterial communities are highly relevant to the digestion, nutrition, growth, reproduction, and a range of fitness in fish, but little is known about the gut microbial community in Antarctic fish. In this study, the composition of intestinal microbial community in four species of Antarctic fish was detected based on 16S rRNA gene sequencing. As a result, 1 004 639 sequences were obtained from 13 samples identified into 36 phyla and 804 genera, in which Proteobacteria, Actinobacteria, Firmicutes, Thermi, and Bacteroidetes were the dominant phyla, and Rhodococcus, Thermus, Acinetobacter, Propionibacterium, Streptococcus, and Mycoplasma were the dominant genera. The number of common OTUs (operational taxonomic units) varied from 346 to 768, while unique OTUs varied from 84 to 694 in the four species of Antarctic fish. Moreover, intestinal bacterial communities in individuals of each species were not really similar, and those in the four species were not absolutely different, suggesting that bacterial communities might influence the physiological characteristics of Antarctic fish, and the common bacterial communities might contribute to the fish survival ability in extreme Antarctic environment, while the different ones were related to the living habits. All of these results could offer certain information for the future study of Antarctic fish physiological characteristics.

RNA-sequencing of the sturgeon Acipenser baeri provides insights into expression dynamics of morphogenic differentiation and developmental regulatory genes in early versus late developmental stages.

BACKGROUND: Acipenser baeri, one of the critically endangered animals on the verge of extinction, is a key species for evolutionary, developmental, physiology and conservation studies and a standout amongst the most important food products worldwide. Though the transcriptome of the early development of A. baeri has been published recently, the transcriptome changes occurring in the transition from embryonic to late stages are still unknown. The aim of this work was to analyze the transcriptomes of embryonic and post-embryonic stages of A. baeri and identify differentially expressed genes (DEGs) and their expression patterns using mRNA collected from specimens at big yolk plug, wide neural plate and 64 day old sturgeon developmental stages for RNA-Seq. RESULTS: The paired-end sequencing of the transcriptome of samples of A. baeri collected at two early (big yolk plug (T1, 32 h after fertilization) and wide neural plate formation (T2, 45 h after fertilization)) and one late (T22, 64 day old sturgeon) developmental stages using Illumina Hiseq2000 platform generated 64039846, 64635214 and 75293762 clean paired-end reads for T1, T2 and T22, respectively. After quality control, the sequencing reads were de novo assembled to generate a set of 149,265 unigenes with N50 value of 1277 bp. Functional annotation indicated that a substantial number of these unigenes had significant similarity with proteins in public databases. Differential expression profiling allowed the identification of 2789, 12,819 and 10,824 DEGs from the respective T1 vs. T2, T1 vs. T22 and T2 vs. T22 comparisons. High correlation of DEGs' features was recorded among early stages while significant divergences were observed when comparing the late stage with early stages. GO and KEGG enrichment analyses revealed the biological processes, cellular component, molecular functions and metabolic pathways associated with identified DEGs. The qRT-PCR performed for candidate genes in specimens confirmed the validity of the RNA-seq data. CONCLUSIONS: This study presents, for the first time, an extensive overview of RNA-Seq based characterization of the early and post-embryonic developmental transcriptomes of A. baeri and provided 149,265 gene sequences that will be potentially valuable for future molecular and genetic studies in A. baeri.

The complete mitochondrial genome sequence and gene organization of Trematomus bernacchii (Perciformes: Nototheniidae) with phylogenetic consideration.

In this study, the complete mitochondrial DNA sequence was obtained from Trematomus bernacchii. The full length of mitogenome was 16 018 bp, including 20 transfer RNA genes, a 16S ribosomal RNA genes and an incomplete 12S rRNA, 12 protein-coding genes and an incomplete ND6 protein-coding gene, and 2 non-coding regions: control region (D-loop) and origin of light-strand (L-strand) replication (OL). Most genes were located on the heavy-strand (H-strand), while ND6 and seven tRNA genes were located on the L-strand. T. bernacchii's mitogenome was comprised of 24.52% for A, 25.44% for C, 20.38% for G and 29.66% for T, with a higher A + T content (54.18%). From the NJ phylogenetic tree, it revealed that T. bernacchii's was genetically closest to species Dissostichus eleginoides among seven species within suborder Notothenioidei. This study could lay the foundation for the future studies in species identification, taxonomic status, molecular systematics, stock evaluation and conservation genetics for T. bernacchii's.

The complete mitochondrial genome of Chionodraco hamatus (Notothenioidei: Channichthyidae) with phylogenetic consideration.

The complete mitochondrial genome of Chionodraco hamatus was obtained, which was 17 457 bp in length. This genome consists of 13 protein-coding genes, 22 transfer RNA genes, 2 ribosomal RNA genes and a putative control region. Of the 37 genes, 28 were encoded by heavy strand, while 9 were encoded by light strand. Overall base composition of mitogenome is estimated to be 26.38% for A, 17.44% for G, 26.00% for T, 30.18% for C, respectively, with a slight A + T bias (52.38%). The phylogenetic analysis based on 13 concatenated protein-coding genes suggested that C. hamatus as a sister species to Chionodraco myersi was clustered in family Chionodraco. The complete mitochondrial genome sequence of C. hamatus could provide a basic data for the studies on evolution for low temperature adaptability, population structure, molecular systematic, stock evaluation and conservation genetics.

Two transcripts of HMG-CoA reductase related with developmental regulation from Scylla paramamosain: Evidences from cDNA cloning and expression analysis.

3-Hydroxy-3-methylglutaryl coenzyme-A (HMG-CoA) reductases (HMGRs), which catalyze the conversion of HMG-CoA to mevalonate, may have an important role in the synthesis of methyl farnesoate (MF). In this study, we obtained two HMGR cDNA sequences termed Sp-HMGR1 (membrane-bound form) and Sp-HMGR2 (soluble form), which encode 967 and 654 amino acids, respectively. The two cDNAs possess entirely identical sequences except that Sp-HMGR1 is 1,382 bp, which encodes a sterol-sensed domain (SSD; a membrane-bound domain) and was first found in crustacean HMGR, larger than Sp-HMGR2. Thus, it was deduced that these cDNAs might be derived from a single genomic DNA sequence. Sp-HMGRs have the typical features of the HMGR class of proteins. However, residue 844 in Sp-HMGR1, which is usually occupied by a Ser residue in other species, has an unusual Ala substitution. This Ser is thought to be involved in enzyme activity regulation by reversible phosphorylation. A putative "PEST" sequence that, until now, has only been found in crustacean species was also identified in the C-terminus of both transcripts, and a sterol-sensing domain, which was first found in crustacean species, was identified in Sp-HMGR1; these findings suggest that Sp-HMGR might function in some special regulatory mechanism. Furthermore, the quantitative real-time polymerase chain reaction results showed that the two transcripts have different expression patterns; Sp-HMGR2 was mainly expressed in the mandibular organ (MO) of adult crabs, whereas Sp-HMGR1 was mainly expressed in other tissues and fertilized eggs up until the fourth juvenile crab stage. The fluctuating gene expression seemed to suggest a relationship between Sp-HMGRs and the development of the crab, especially during the larval stage. Besides, the fluctuation of Sp-HMGR1 in ovary, brain, and thoracic ganglia during the ovary development seemed to have some correlation with the nutrition accumulation of ovaries, whether the SSD domain evolved in this process deserve further investigation. Moreover, it remains unclear whether the significant variation in ovary, brain, and thoracic ganglia during ovary development suggests that other tissues in addition to the MO could synthesize MF.

Transcriptome Sequencing, De Novo Assembly and Differential Gene Expression Analysis of the Early Development of Acipenser baeri.

The molecular mechanisms that drive the development of the endangered fossil fish species Acipenser baeri are difficult to study due to the lack of genomic data. Recent advances in sequencing technologies and the reducing cost of sequencing offer exclusive opportunities for exploring important molecular mechanisms underlying specific biological processes. This manuscript describes the large scale sequencing and analyses of mRNA from Acipenser baeri collected at five development time points using the Illumina Hiseq2000 platform. The sequencing reads were de novo assembled and clustered into 278167 unigenes, of which 57346 (20.62%) had 45837 known homologues proteins in Uniprot protein databases while 11509 proteins matched with at least one sequence of assembled unigenes. The remaining 79.38% of unigenes could stand for non-coding unigenes or unigenes specific to A. baeri. A number of 43062 unigenes were annotated into functional categories via Gene Ontology (GO) annotation whereas 29526 unigenes were associated with 329 pathways by mapping to KEGG database. Subsequently, 3479 differentially expressed genes were scanned within developmental stages and clustered into 50 gene expression profiles. Genes preferentially expressed at each stage were also identified. Through GO and KEGG pathway enrichment analysis, relevant physiological variations during the early development of A. baeri could be better cognized. Accordingly, the present study gives insights into the transcriptome profile of the early development of A. baeri, and the information contained in this large scale transcriptome will provide substantial references for A. baeri developmental biology and promote its aquaculture research.

Miro1 deficiency in amyotrophic lateral sclerosis.

Proper transportation of mitochondria to sites with high energy demands is critical for neuronal function and survival. Impaired mitochondrial movement has been repeatedly reported in motor neurons of amyotrophic lateral sclerosis (ALS) patients and indicated as an important mechanism contributing to motor neuron degeneration in ALS. Miro1, a RhoGTPase also referred to as Rhot1, is a key regulator of mitochondrial movement linking mitochondria and motor proteins. In this study, we investigated whether the expression of Miro1 was altered in ALS patients and ALS animal models. Immunoblot analysis revealed that Miro1 was significantly reduced in the spinal cord tissue of ALS patients. Consistently, the decreased expression of Miro1 was also noted only in the spinal cord, and not in the brain tissue of transgenic mice expressing ALS-associated SOD1 G93A or TDP-43 M337V. Glutamate excitotoxicity is one of the major pathophysiological mechanisms implicated in the pathogenesis of ALS, and we found that excessive glutamate challenge lead to significant reduction of Miro1 expression in spinal cord motor neurons both in vitro and in mice. Taken together, these findings show Miro1 deficiency in ALS patients and ALS animal models and suggest glutamate excitotoxicity as a likely cause of Miro1 deficiency.