The Influence of Fermentation TIME in the Physical and Chemical Composition of Fermented Soybean Husk by Using Aspergillus Niger on the Quality of Raw Feed Materials

Soybean husk (Glycine max L. Merrill) a soybean processing waste as raw material for tempe obtained after the process of boiling and soaking soybeans. The main problem in the use of soybean husk (Glycine max L. Merrill) as feed material is its crude fiber content which is fairly high. This study aimed to observe the fermented soybean husk using Aspergillus Niger to improve the quality of the raw feed materials. This was conducted by using completely randomized design (CRD) analysis and repeated three times; the time optimization of Aspergillus Niger in 2, 4, and 6 days based on chemical analyses (moisture, protein, fat, ash, crude fiber and feed containing carbohydrates (NFE) and physical assessment fermentation (smell, texture, moisture and hyphae) were analyzed descriptive qualitatively. The results showed that 4 days fermentation of soybean husk using A. Niger is successful gives the highest score based on physical characteristics texture, aroma, moisture, and the formed hyphae and the most effective treatment for decrease in crude fiber is 13% and increase in NFE contained in the largest on 4 days fermented soybean husk by Aspergillus Niger with a long time 4 days

Dried Skeletonema Costatum in Feed Formulation for the Growth of Vaname Shrimp (Litopenaeus Vannamei)

The aim of this study was to determine the effect and the best dosage used by the Skeletonema costatum in feed formulation on the growth of Vaname shrimp. This experiment used Completely Randomized Design (CRD) with four treatments and three replications. Treatment was given by using dried S. costatum in feed formula (iso protein 37% and iso energy 3.6 kkal/g feed). Feed treatment was the substitution of fish meal protein A = 0%, B = 2.5%, C = 5% and D = 7.5% with dried S. costatum protein. The observed parameters include Survival Rate (SR), Survival Growth Rate (SGR), Feed Efficiency Ratio (FER), and Protein Efficiency Ratio (PER). The results showed that the use of dried S. costatum in feeding are effective in increasing the specific growth rate, feed efficiency ratio and the ratio of protein efficiency than controls. The best dosage in feeding formula is ranged from 4.39% to 4.75%

Plasma S-adenosylmethionine, DNMT polymorphisms, and peripheral blood LINE-1 methylation among healthy Chinese adults in Singapore

10.1186/1471-2407-13-389BMC Cancer13-BCMA

Ataxia with oculomotor apraxia type 2: clinical, biological and genotype/phenotype correlation study of a cohort of 90 patients

Ataxia with oculomotor apraxia type 2 (AOA2) is an autosomal recessive disease due to mutations in the senataxin gene, causing progressive cerebellar ataxia with peripheral neuropathy, cerebellar atrophy, occasional oculomotor apraxia and elevated alpha-feto-protein (AFP) serum level. We compiled a series of 67 previously reported and 58 novel ataxic patients who underwent senataxin gene sequencing because of suspected AOA2. An AOA2 diagnosis was established for 90 patients, originating from 15 countries worldwide, and 25 new senataxin gene mutations were found. In patients with AOA2, median AFP serum level was 31.0 mu g/l at diagnosis, which was higher than the median AFP level of AOA2 negative patients: 13.8 mu g/l, P = 0.0004; itself higher than the normal level (3.4 mu g/l, range from 0.5 to 17.2 mu g/l) because elevated AFP was one of the possible selection criteria. Polyneuropathy was found in 97.5% of AOA2 patients, cerebellar atrophy in 96%, occasional oculomotor apraxia in 51%, pyramidal signs in 20.5%, head tremor in 14%, dystonia in 13.5%, strabismus in 12.3% and chorea in 9.5%. No patient was lacking both peripheral neuropathy and cerebellar atrophy. The age at onset and presence of occasional oculomotor apraxia were negatively correlated to the progression rate of the disease (P = 0.03 and P = 0.009, respectively), whereas strabismus was positively correlated to the progression rate (P = 0.03). An increased AFP level as well as cerebellar atrophy seem to be stable in the course of the disease and to occur mostly at or before the onset of the disease. One of the two patients with a normal AFP level at diagnosis had high AFP levels 4 years later, while the other had borderline levels. The probability of missing AOA2 diagnosis, in case of sequencing senataxin gene only in non-Friedreich ataxia non-ataxia-telangiectasia ataxic patients with AFP level >= 7 mu g/l, is 0.23% and the probability for a non-Friedreich ataxia non-ataxia-telangiectasia ataxic patient to be affected with AOA2 with AFP levels >= 7 mu g/l is 46%. Therefore, selection of patients with an AFP level above 7 mu g/l for senataxin gene sequencing is a good strategy for AOA2 diagnosis. Pyramidal signs and dystonia were more frequent and disease was less severe with missense mutations in the helicase domain of senataxin gene than with missense mutations out of helicase domain and deletion and nonsense mutations (P = 0.001, P = 0.008 and P = 0.01, respectively). The lack of pyramidal signs in most patients may be explained by masking due to severe motor neuropathy

NMDA receptor gene variations as modifiers in Huntington disease: a replication study.

Several candidate modifier genes which, in addition to the pathogenic CAG repeat expansion, influence the age at onset (AO) in Huntington disease (HD) have already been described. The aim of this study was to replicate association of variations in the N-methyl D-aspartate receptor subtype genes GRIN2A and GRIN2B in the "REGISTRY" cohort from the European Huntington Disease Network (EHDN). The analyses did replicate the association reported between the GRIN2A rs2650427 variation and AO in the entire cohort. Yet, when subjects were stratified by AO subtypes, we found nominally significant evidence for an association of the GRIN2A rs1969060 variation and the GRIN2B rs1806201 variation. These findings further implicate the N-methyl D-aspartate receptor subtype genes as loci containing variation associated with AO in HD

Bi-allelic JAM2 Variants Lead to Early-Onset Recessive Primary Familial Brain Calcification.

Primary familial brain calcification (PFBC) is a rare neurodegenerative disorder characterized by a combination of neurological, psychiatric, and cognitive decline associated with calcium deposition on brain imaging. To date, mutations in five genes have been linked to PFBC. However, more than 50% of individuals affected by PFBC have no molecular diagnosis. We report four unrelated families presenting with initial learning difficulties and seizures and later psychiatric symptoms, cerebellar ataxia, extrapyramidal signs, and extensive calcifications on brain imaging. Through a combination of homozygosity mapping and exome sequencing, we mapped this phenotype to chromosome 21q21.3 and identified bi-allelic variants in JAM2. JAM2 encodes for the junctional-adhesion-molecule-2, a key tight-junction protein in blood-brain-barrier permeability. We show that JAM2 variants lead to reduction of JAM2 mRNA expression and absence of JAM2 protein in patient's fibroblasts, consistent with a loss-of-function mechanism. We show that the human phenotype is replicated in the jam2 complete knockout mouse (jam2 KO). Furthermore, neuropathology of jam2 KO mouse showed prominent vacuolation in the cerebral cortex, thalamus, and cerebellum and particularly widespread vacuolation in the midbrain with reactive astrogliosis and neuronal density reduction. The regions of the human brain affected on neuroimaging are similar to the affected brain areas in the myorg PFBC null mouse. Along with JAM3 and OCLN, JAM2 is the third tight-junction gene in which bi-allelic variants are associated with brain calcification, suggesting that defective cell-to-cell adhesion and dysfunction of the movement of solutes through the paracellular spaces in the neurovascular unit is a key mechanism in CNS calcification

Outlier detection and classification in sensor data streams for proactive decision support systems

A paper has a deal with the problem of quality assessment in sensor data streams accumulated by proactive decision support systems. The new problem is stated where outliers need to be detected and to be classified according to their nature of origin. There are two types of outliers defined; the first type is about misoperations of a system and the second type is caused by changes in the observed system behavior due to inner and external influences. The proposed method is based on the data-driven forecast approach to predict the values in the incoming data stream at the expected time. This method includes the forecasting model and the clustering model. The forecasting model predicts a value in the incoming data stream at the expected time to find the deviation between a real observed value and a predicted one. The clustering method is used for taxonomic classification of outliers. Constructive neural networks models (CoNNS) and evolving connectionists systems (ECS) are used for prediction of sensors data. There are two real world tasks are used as case studies. The maximal values of accuracy are 0.992 and 0.974, and F1 scores are 0.967 and 0.938, respectively, for the first and the second tasks. The conclusion contains findings how to apply the proposed method in proactive decision support systems

Biallelic mutations in neurofascin cause neurodevelopmental impairment and peripheral demyelination.

Axon pathfinding and synapse formation are essential processes for nervous system development and function. The assembly of myelinated fibres and nodes of Ranvier is mediated by a number of cell adhesion molecules of the immunoglobulin superfamily including neurofascin, encoded by the NFASC gene, and its alternative isoforms Nfasc186 and Nfasc140 (located in the axonal membrane at the node of Ranvier) and Nfasc155 (a glial component of the paranodal axoglial junction). We identified 10 individuals from six unrelated families, exhibiting a neurodevelopmental disorder characterized with a spectrum of central (intellectual disability, developmental delay, motor impairment, speech difficulties) and peripheral (early onset demyelinating neuropathy) neurological involvement, who were found by exome or genome sequencing to carry one frameshift and four different homozygous non-synonymous variants in NFASC. Expression studies using immunostaining-based techniques identified absent expression of the Nfasc155 isoform as a consequence of the frameshift variant and a significant reduction of expression was also observed in association with two non-synonymous variants affecting the fibronectin type III domain. Cell aggregation studies revealed a severely impaired Nfasc155-CNTN1/CASPR1 complex interaction as a result of the identified variants. Immunofluorescence staining of myelinated fibres from two affected individuals showed a severe loss of myelinated fibres and abnormalities in the paranodal junction morphology. Our results establish that recessive variants affecting the Nfasc155 isoform can affect the formation of paranodal axoglial junctions at the nodes of Ranvier. The genetic disease caused by biallelic NFASC variants includes neurodevelopmental impairment and a spectrum of central and peripheral demyelination as part of its core clinical phenotype. Our findings support possible overlapping molecular mechanisms of paranodal damage at peripheral nerves in both the immune-mediated and the genetic disease, but the observation of prominent central neurological involvement in NFASC biallelic variant carriers highlights the importance of this gene in human brain development and function