Low Level of Dietary Organic Trace Elements Improve the Eggshell Strength, Trace Element Utilization, and Intestinal Function in Late-Phase Laying Hens.

This study was conducted to evaluate the effects of organic trace elements (Cu, Fe, Zn, and Mn) on performance, egg quality, trace elements utilization, and intestinal function in late-phase laying hens. A total of 1,080 laying hens (Hy-line brown, 65 weeks old) were randomly assigned to four treatments with six replications of 45 layers each. The basal diet was prepared without adding exogenous trace elements. The control group was fed with a basal diet supplemented with 600 mg/kg of inorganic trace elements. The three treatment groups were fed basal diets supplemented with 300, 450, and 600 mg/kg organic trace elements (OTE300, 450, and 600), respectively. The results showed that there was no significant difference in growth performance among all treatments. However, OTE450 significantly improved the eggshell strength of laying hens (p < 0.05), but had no significant effects on haugh unit, egg yolk weight, eggshell weight, and eggshell thickness, compared with other groups. Moreover, compared with the control group, OTE450 significantly increased the contents of copper, iron, and zinc in serum (p < 0.05). Meanwhile, all of the trace elements had a lower deposition in the feces in organic trace elements groups (p < 0.05). Histological analysis showed that the addition of organic trace elements could significantly improve the villus height and villus concealment ratio (p < 0.05). In addition, the messenger RNA (mRNA) and protein expressions of divalent metal transporter 1 (DMT1), zinc transporter 1 (ZnT-1), and ferroportin 1 (FPN1) were the highest in the OTE450 group. In conclusion, OTE450 could improve egg quality, intestinal function, and trace element utilization efficiency. Thus, this study provides a theoretical basis for the application of low levels of organic trace elements in laying hens.

Life extension factor klotho regulates behavioral responses to stress via modulation of GluN2B function in the nucleus accumbens.

Klotho is a life extension factor that has the ability to regulate the function of GluN2B-containing N-methyl-D-aspartate receptors (NMDARs), whose dysfunction in the nucleus accumbens (NAc) underlies critical aspects of the pathophysiology of major depression. Here, we study the functional relevance of klotho in the pathogenesis of depression. A chronic social defeat stress paradigm, in which mice are categorized as either susceptible or unsusceptible based on their performance in a social interaction test, was used in this study. We found that the expression of klotho was largely decreased in the NAc of susceptible mice compared to control or unsusceptible mice. Genetic knockdown of klotho in the NAc induced behavioral alterations relevant to depression in naive mice, while overexpression of klotho produced an antidepressive effect in normal mice and ameliorated the behavioral responses to stress in susceptible mice. Molecularly, knockdown of klotho in the NAc resulted in selective decreases in total and synaptic GluN2B expression that were identical to those in susceptible mice. Elevation of klotho in the NAc reversed the reductions in GluN2B expressions and altered synaptic transmission and spine density in the NAc of susceptible mice. Furthermore, blockade of GluN2B with a specific antagonist abolished the beneficial effects of klotho elevation in susceptible mice. Collectively, we demonstrated that klotho in the NAc modulates behavioral responses to stress by regulating the function of GluN2B-containing NMDARs. These results reveal a novel role for klotho in the pathogenesis of depression, providing new insights into the molecular basis of major depression.

Decreased Plasma Hydrogen Sulfide Level Is Associated With the Severity of Depression in Patients With Depressive Disorder.

Accumulating evidence has suggested a dysfunction of synaptic plasticity in the pathophysiology of depression. Hydrogen sulfide (H2S), an endogenous gasotransmitter that regulates synaptic plasticity, has been demonstrated to contribute to depressive-like behaviors in rodents. The current study investigated the relationship between plasma H2S levels and the depressive symptoms in patients with depression. Forty-seven depressed patients and 51 healthy individuals were recruited in this study. The 17-item Hamilton Depression Rating Scale (HAMD-17) was used to evaluate depressive symptoms for all subjects and the reversed-phase high-performance liquid chromatography (RP-HPLC) was used to measure plasmaH2S levels. We found that plasma H2S levels were significantly lower in patients with depression relative to healthy individuals (P < 0.001). Compared with healthy controls (1.02 ± 0.34 µmol/L), the plasma H2S level significantly decreased in patients with mild depression (0.84 ± 0.28 µmol/L), with moderate depression (0.62 ± 0.21µmol/L), and with severe depression (0.38 ± 0.18 µmol/L). Correlation analysis revealed that plasma H2S levels were significantly negatively correlated with the HAMD-17 scores in patients (r = -0.484, P = 0.001). Multivariate linear regression analysis showed that plasma H2S was an independent contributor to the HAMD-17 score in patients (B = -0.360, t = -2.550, P = 0.015). Collectively, these results suggest that decreased H2S is involved in the pathophysiology of depression, and plasma H2S might be a potential indicator for depression severity.

The complete chloroplast genome sequence of Alniphyllum fortunei (Styracaceae) from Fujian, China.

Alniphyllum fortunei is a subtropical tree species, a large deciduous tree with a tall and straight trunk, which is an excellent fast-growing and broad-leaved tree species with a wide range of uses we resequenced complete chloroplast (cp) genome of A. fortunei from Fujian, China. The whole genome was 154,166 bp in length, consisting of a pair of inverted repeats (IR 26,658 bp), a large single-copy region (LSC 82,438 bp), and a small single-copy region (SSC 18,367 bp). The complete genome contained 139 genes, including 89 protein-coding genes, 40 tRNA, and 8 rRNA genes. The phylogenetic analyses based on the complete chloroplast genome sequence provided solid evidence that A. fortunei has a close relationship with A. pterospermum and Bruinsmia polysperma.

Zearalenone regulates key factors of the Kelch-like erythroid cell-derived protein with CNC homology-associated protein 1-nuclear factor erythroid 2-related factor 2 signaling pathway in duodenum of post-weaning gilts.

OBJECTIVE: This study explored the mechanism of the Kelch-like erythroid cell-derived protein with CNC homology-associated protein 1 (Keap1)-nuclear factor erythroid 2-related factor 2 (Nrf2) signaling pathway under conditions of zearalenone (ZEA)-induced oxidative stress in the duodenum of post-weaning gilts. METHODS: Forty post-weaning gilts were randomly allocated to four groups and fed diets supplemented with 0, 0.5, 1.0, or 1.5 mg/kg ZEA. RESULTS: The results showed significant reductions in the activity of the antioxidant enzymes total superoxide dismutase and glutathione peroxidase and increases the malondialdehyde content with increasing concentrations of dietary ZEA. Immunohistochemical analysis supported these findings by showing a significantly increased expression of Nrf2 and glutathione peroxidase 1 (GPX1) with increasing concentrations of ZEA. The relative mRNA and protein expression of Nrf2, GPX1 increased linearly (p<0.05) and quadratically (p<0.05), which was consistent with the immunohistochemical results. The relative mRNA expression of Keap1 decreased linearly (p<0.05) and quadratically (p<0.05) in the duodenum as the ZEA concentration increased in the diet. The relative mRNA expression of modifier subunit of glutamate-cysteine ligase (GCLM) increased quadratically (p<0.05) in all ZEA treatment groups and the relative mRNA expression of quinone oxidoreductase 1 (NQO1) catalytic subunit of glutamate-cysteine ligase decreased linearly (p<0.05) and quadratically (p<0.05) in the ZEA1.0 group and ZEA1.5 group. The relative protein expression of Keap1 and GCLM decreased quadratically (p<0.05) in the duodenum as the ZEA concentration increased in the diet, respectively. The relative protein expression of NQO1 increased linearly (p<0.05) and quadratically (p<0.05) in all ZEA treatment groups in the duodenum. CONCLUSION: These findings suggest that ZEA regulates the expression of key factors of the Keap1-Nrf2 signaling pathway in the duodenum, which enables resistance to ZEA-induced oxidative stress. Further studies are needed to examine the effects of ZEA induced oxidative stress on other tissues and organs in post-weaning gilts.

Erratum to: Changes of Serum Insulin-like Growth Factor-2 Response to Negative Symptom Improvements in Schizophrenia Patients Treated with Atypical Antipsychotics.

The article "Changes of Serum Insulin-like Growth Factor-2 Response to Negative Symptom Improvements in Schizophrenia Patients Treated with Atypical Antipsychotics", written by Xue-lin CHAO, Shu-zhen JIANG, Jian-wen XIONG, Jin-qiong ZHAN, Bo WEI, Chun-nuan CHEN, Yuan-jian YANG was originally published electronically on the publisher's internet portal on June 2020 without open access. With the author(s)' decision to opt for Open Choice, the copyright of the article is changed to © The Author(s) 2020 and the article is forthwith distributed under a Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/), which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

The Phoebe genome sheds light on the evolution of magnoliids.

Lauraceae includes the genus Phoebe, and the family is linked to the evolution of magnoliids. We sequenced the genome of Phoebe bournei Nanmu. The assembled genome size was 989.19 Mb, with a contig N50 value of 2.05 Mb. A total of 28,198 protein-coding genes were annotated in P. bournei. Whole-genome duplication (WGD) analysis showed that Lauraceae has experienced two WGD events; the older WGD event occurred just before the divergence of Lauraceae and Magnoliales, and the more recent WGD was shared by all lineages of Lauraceae. The phylogenetic tree showed that magnoliids form a sister clade to monocots and eudicots. We also identified 63 MADS-box genes, including AGL12-like genes that may be related to the regulation of P. bournei roots and FIN219-like genes encoding GH3 proteins, which are involved in photomorphogenesis. SAUR50-like genes involved in light signal-mediated pedicel or stem development were also identified. Four ATMYB46- and three PtrEPSP-homologous genes related to lignin biosynthesis were identified. These genes may be associated with the formation of straight trunks in P. bournei. Overall, the P. bournei reference genome provides insight into the origin, evolution, and diversification of Phoebe and other magnoliids.

Changes of Serum Insulin-like Growth Factor-2 Response to Negative Symptom Improvements in Schizophrenia Patients Treated with Atypical Antipsychotics.

Accumulating evidence suggests that a disruption of early brain development, in which insulin-like growth factor-2 (IGF-2) has a crucial role, may underlie the pathophysiology of schizophrenia. Our previous study has shown that decreased serum IGF-2 was correlated with the severity of psychopathology in patients with schizophrenia. Here we conducted a prospective observation trial to investigate the effects of atypical antipsychotics on serum IGF-2 level and its relationship with clinical improvements in schizophrenia patients. Thirty-one schizophrenia patients with acute exacerbation and 30 healthy individuals were recruited in this study. Psychiatric symptoms were assessed using the Positive and Negative Syndrome Scale (PANSS) and serum IGF-2 levels were determined using ELISA. We found that schizophrenia patients with acute exacerbation had lower serum IGF-2 levels than control individuals at baseline (P<0.05). After 2 months of atypical antipsychotic treatment, a significant improvement in each PANSS subscore and total score was observed in patients (all P<0.01), and the serum IGF-2 levels of patients were significantly increased compared with those at baseline (203.13±64.62 vs. 426.99±124.26 ng/mL; t =-5.044, P<0.001). Correlation analysis revealed that the changes of serum IGF-2 levels in patients were significantly correlated with the improvements of negative symptoms (r=-0.522, P=0.006). Collectively, our findings demonstrated changes of serum IGF-2 response to improvements of negative symptoms in schizophrenia patients treated with atypical antipsychotics, suggesting that serum IGF-2 might be a treatment biomarker for schizophrenia.

Altered insulin-like growth factor-2 signaling is associated with psychopathology and cognitive deficits in patients with schizophrenia.

BACKGROUND: Schizophrenia is linked with abnormal brain neurodevelopment, on which IGF-2 (insulin-like growth factor-2) has a great impact. The purpose of this study was to assess the levels of serum IGF-2 and its binding proteins IGFBP-3 and IGFBP-7 in schizophrenia patients and the associations of these proteins with schizophrenia psychopathology and cognitive deficits. METHODS: Thirty-two schizophrenia patients and 30 healthy controls were recruited. The PANSS and a neurocognitive test battery were used to assess schizophrenic symptomatology and cognition, respectively. Serum IGF-2, IGFBP-3 and IGFBP-7 levels were determined using ELISA. RESULTS: The schizophrenia patients had a much lower content of serum IGF-2, IGFBP-3 and IGFBP-7 than controls. For the patients, IGF-2 levels were negatively correlated with the PANSS negative scores and positively associated with working memory, attention, and executive function. The correlations between IGF-2 and the PANSS negative scores, working memory or executive function were still significant after controlling for age, sex, education level, BMI, illness history and age of onset. No significant associations of IGFBP-3 or IGFBP-7 with the PANSS scores and cognitive function were observed in the patients. CONCLUSIONS: Our study demonstrates that serum IGF-2 was significantly correlated with negative and cognitive symptoms in patients with schizophrenia, suggesting that altered IGF-2 signaling may be implicated in the psychopathology and cognitive deficits in schizophrenia.

The complete chloroplast genome sequence of Acorus tatarinowii (Araceae) from Fujian, China.

Acorus tatarinowii is a useful traditional Chinese medicine (TCM) and is officially documented in the Chinese Pharmacopeia with the name 'Shi Chang Pu'. It belongs to the Araceae family and is used for the treatment of dementia, epilepsy, amnesia and insomnia. We resequenced complete chloroplast (cp) genome of A. tatarinowii from Fujian, China. The whole genome was 153,453 bp in length, consisting of a pair of inverted repeats (IR 25,795 bp), a large single-copy region (LSC 83,631 bp), and a small single-copy region (SSC 18,232 bp). The complete genome contained 132 genes, including 84 protein-coding genes, 38 tRNA, and 8 rRNA genes. The overall GC content of the whole genome was 38.7%. A maximum-likelihood phylogenetic analysis showed that A. tatarinowii is sister to A. tatarinowii which was collected in Yunnan, China. The complete chloroplast genome of A. tatarinowii will help improve and integrate the existing genome data of monocots and provide insights into the phylogenetic relationship among basal angiosperms, monocots and dicots.

The association between serum insulin-like growth factor 1 and cognitive impairments in patients with schizophrenia.

Accumulating evidence has shown that insulin-like growth factors (IGFs) are implicated in schizophrenia. Altered serum levels of IGF-1 have been found in schizophrenia patients and are associated with psychopathological symptoms. However, whether there is a relationship between IGF-1 and cognitive impairment in schizophrenia remains unknown. Thirty schizophrenia patients and 26 healthy controls were recruited for this study. The Positive and Negative Syndrome Scale was adopted to assess schizophrenic symptoms, and a battery of neuropsychological tests was employed to evaluate cognitive function. Serum IGF-1 content was determined by enzyme-linked immunosorbent assay (ELISA). We found that patients with schizophrenia performed more poorly than healthy controls in most cognitive tasks, excluding visual memory. The serum IGF-1 concentrations in schizophrenia patients were much lower than those in controls. Correlation analyses revealed that the levels of serum IGF-1 were positively correlated with executive function and attention scores in patients. Furthermore, IGF-1 was an independent contributor to deficits in executive function and attention among schizophrenia patients. Collectively, serum IGF-1 levels were significantly correlated with cognitive performance in schizophrenia patients, indicating that decreased IGF-1 levels might contribute to the pathophysiology of schizophrenia-associated cognitive impairments. The regulation of IGF-1 signaling might be a potential treatment strategy for cognitive impairments in schizophrenia.

Zearalenone-Promoted Follicle Growth through Modulation of Wnt-1/ß-Catenin Signaling Pathway and Expression of Estrogen Receptor Genes in Ovaries of Postweaning Piglets.

Feedstuffs are severely contaminated by zearalenone (ZEA) worldwide. A specific dietary level of ZEA could cause malformations of the reproductive organs of sows, false estrus, decreased litter size, and abortion. However, the underlying mechanisms are still not clear. The objectives of the present study were to assess the effects of ZEA on morphology, distribution, and expression of estrogen receptors (ERα and ERß) in the ovaries of postweaning piglets. Furthermore, the relationship between ERs/glycogen synthase kinase (GSK)-3ß-dependent pathways mediated by ZEA and the Wnt-1/ß-catenin signaling pathway was examined. Forty healthy weaning piglets were allocated to the following four treatment groups: piglets fed with basal diet only (control), and ZEA0.5, ZEA1.0, and ZEA1.5, which were fed basal diets supplemented with ZEA at 0.5, 1.0, and 1.5 mg·kg-1, respectively. Then, the expression of GSK-3ß, ERα, ERß, and Wnt-1/ß-catenin were examined histomorphologically and immunohistochemically. Results showed that the proportion of primordial follicles (PrF's) decreased ( p < 0.001) but that of atretic primordial follicles (APFs) increased ( p < 0.001) with increasing dietary ZEA levels. More interestingly, the immunopositivity of ERß in the ovaries was stronger than that of ERα with the same treatment. The relative mRNA and protein expression levels of ERα, ERß, Wnt-1, ß-catenin, and GSK-3ß in the ovaries of postweaning gilts increased linearly ( p < 0.05) as dietary ZEA concentrations increased. Moreover, the accumulation of Wnt-1 and ß-catenin in the ovaries indicated that ZEA activated the Wnt-1/ß-catenin pathway, mediated by ERs/GSK-3ß. Our results strongly suggested that ovarian follicles in the ZEA (0.5-1.5 mg·kg-1)-treated groups were highly proliferative state, indicating that ZEA promoted ovarian development. The results also suggested that ZEA activates the ERs/GSK-3ß-dependent Wnt-1/ß-catenin signaling pathway, indicating its important role in accelerating development of the ovaries.

Decreased plasma levels of gasotransmitter hydrogen sulfide in patients with schizophrenia: correlation with psychopathology and cognition.

OBJECTIVE: Aberrant N-methyl-D-aspartate receptor (NMDAR) function has been implicated in the pathophysiology of schizophrenia. Hydrogen sulfide (H2S) is an endogenous gasotransmitter that regulates NMDAR function. The current study investigated the relationship between plasma H2S levels and both psychopathological and cognitive symptoms in schizophrenia. MATERIALS AND METHODS: Forty-one patients with schizophrenia and 40 healthy control subjects were recruited in present study. Schizophrenic symptomatology was assessed using the Positive and Negative Syndrome Scale (PANSS). Cognitive function was evaluated with a neuropsychological battery including seven neurocognitive tests. Plasma H2S levels were measured by reversed-phase high-performance liquid chromatography (RP-HPLC). RESULTS: Patients with schizophrenia performed worse in all of the cognitive tests than the healthy controls except for the visual memory. Plasma H2S levels were significantly lower in patients with schizophrenia relative to healthy control subjects (F = 3.821, p = 0.007). Correlation analysis revealed a significant negative correlation between the H2S levels and the PANSS general scores (r = - 0.413, p = 0.007). Additionally, a positive association was observed between plasma H2S levels and working memory (r = 0.416, p = 0.007), visual memory (r = 0.363, p = 0.020), or executive function (r = 0.344, p = 0.028) in patients. Partial correlation analysis showed that the correlations between the H2S levels and the PANSS general scores, working memory, visual memory, or executive function were still significant when controlling for age, gender, years of education, BMI, duration of illness, and age of onset. CONCLUSION: The significant relations observed in the current study between H2S and the general psychopathological as well as cognitive symptoms suggest that decreased H2S is involved in the psychopathology and cognitive deficits of schizophrenia, and it might be a promising peripheral biomarker of schizophrenia.

Effects of zearalenone on the localization and expression of the growth hormone receptor gene in the uteri of post-weaning piglets.

OBJECTIVE: In this study, we investigated the adverse effects of dietary zearalenone (ZEA) (0.5 to 1.5 mg/kg diet) on the localization and expression of the growth hormone receptor (GHR) in the uteri of post-weaning gilts and explored alternative mechanism of the reproductive toxicity of ZEA on piglets. METHODS: A total of forty healthy piglets (Duroc×Landrace×Large White) aged 28 d were selected for study. Piglets were transferred to single cages after 10 days' adaptation on an obstetric table. The animals were allocated to one of four treatments: a normal basal diet supplemented with 0 (Control), 0.5 (ZEA0.5), 1.0 (ZEA1.0), or 1.5 (ZEA1.5) mg/kg purified ZEA, and fed for 35 d after the 10-d adaptation. Analyzed ZEA concentrations in the diets were 0, 0.52±0.07, 1.04±0.03, and 1.51±0.13 mg/kg, respectively. At the end of the feeding trial, piglets were euthanized after being fasted for 12 h. Two samples of uterine tissue from each pig were rapidly collected, one of which was stored at -80°C for analysis of the relative mRNA and protein expression of GHR, and the second was promptly fixed in Bouin's solution for immunohistochemical analysis. RESULTS: The relative weight of the uteri and thickness of the myometrium and endometrium increased linearly (p<0.001) and quadratically (p<0.001) with an increasing level of ZEA. The results of immunohistochemical analysis indicated that GHR immunoreactive substance was mainly localizated in the cytoplasm of uterine smooth muscle, glandular epithelial, luminal epithelial, stromal, and vascular endothelial cells. In contrast, nuclear staining was rarely observed. The immunoreactive integrated optic density of GHR in the myometrium, luminal epithelium, glandular epithelium, and whole uteri of weaning gilts increased linearly (p<0.001) and quadratically (p<0.05) with an increasing level of ZEA. The mRNA and protein expression of GHR in the uteri of weaning gilts increased linearly (p<0.001) and quadratically (p<0.05) with an increasing level of ZEA. CONCLUSION: In conclusion, ZEA at a concentration of 0.5 mg/kg was sufficient to significantly thicken the myometrium and endometrium, and at a concentration of 1.0 mg/kg induced a high level of GHR expression to promote growth and development of the uteri. This revealed an alternative molecular mechanism whereby ZEA induces growth and development of the uteri and provides a theoretical basis for the revision of Chinese feed hygiene standards.

[Analysis of ALDH5A1 gene mutation in a Chinese Han family with succinic semialdehyde dehydrogenase deficiency].

OBJECTIVE: To detect potential mutation in ALDH5A1 gene for a family affected with succinic semialdehyde dehydrogenase deficiency diagnosed by clinical inspection and urine screening. METHODS: Polymerase chain reaction and direct DNA sequencing were carried out for the affected child and her parents. Suspected ALDH5A1 gene mutations were verified in 100 healthy controls to exclude polymorphisms. RESULTS: The child was found to have carried 2 heterozygous missense mutations in the coding region of ALDH5A1 gene, namely c.527G>A and c.691G>A, for which her mother and father were respectively heterozygotes. The same mutations were not detected in 100 healthy controls. The child was also found to have carried two previously described polymorphisms including a heterozygous c.545C>T(derived from her father) and a homozygous c.538C>T(derived from her mother). CONCLUSION: Missense mutations of c.527G>A and c.691G>A in the ALDH5A1 gene are responsible for the pathogenesis of the disease in this family.

In vitro study of effects of emulsified oil on broiler feed quality.

An experiment was conducted to compare effects of emulsified soybean oil and non-emulsified soybean oil on the quality of broiler feed differing in the feed type and the broiler feeding stage in vitro. A 2 × 2 × 3 factorial arrangement was designed with two fat sources (soybean oil and emulsified oil), two feed types (mash and pellet) and three broiler feeding stages (starter, grower and finisher). Four samples of feeds were collected from each combination of factors at the beginning of the experiment and stored at 20°C. Subsamples were taken at 15-day intervals to determine the moisture content, peroxide value (PV), acid value (AV) and the total fungal count over a 45-day period; weight loss percentage was determined by weighting the samples at days 0, 15, 30 and 45; fines percentage in pellets was only determined at day 0. The emulsified oil reduced (P < 0.05) the fines percentage, increased (P < 0.05) the moisture content, decreased (P < 0.05) the weight loss percentage and PV, did not affect (P > 0.05) the AV and the total fungal count. Results showed that the emulsified oil decreased weight loss, increased pelletability, moisture content and oxidation stability without affecting fungal growth.

[Mutation analysis of a family with 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency].

OBJECTIVE: The aim of this study was to explore the genetic features of a family with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBDD) which may provide the basis for the diagnosis and genetic counseling. METHOD: Clinical data of the proband was collected, total RNA and genomic DNA were extracted from the peripheral blood. The whole coding region of the ACAT1 gene was amplified by RT-PCR. 5' noncoding region of the ACAT1 gene and all 6 exons and flanking intron regions of the HADH2 gene were amplified by PCR. All amplification products were directly sequenced and compared with the reference sequence. RESULT: (1) The patient was a one-year-old boy who presented with psychomotor retardation and astasia when he was admitted to the hospital. Biochemical test revealed slight hyperlactatemia (3.19 mmol/L) and magnetic resonance imaging showed delayed myelination. 2-Methylacetoacetyl-CoA thiolase deficiency was suggested by gas chromatography-mass spectrometry. (2) There was no mutation in the ACAT1 gene and a hemizygous missense mutation c.388C > T was found in the 4 exon of the HADH2 gene which resulted in p. R130C. Proband's mother was the heterozygote and the father was normal. CONCLUSION: This is the first report on MHBDD patient and HADH2 mutation in China. p.R130C is responsible for the pathogenesis of the disease in the infant.

[Effects of Fuzhengyangying granules on the bone marrow proliferation and bcl-2 expression in mice with immune mediated aplastic anemia].

OBJECTIVE: To explore the effect of Fuzhengyangying granules (FZYYG) on the expression of the bone marrow proliferation and bcl-2 in mice with immune mediated aplastic anemia. METHODS: Forty BALB/c mice were randomly allocated to 4 groups: a normal control group, a model group, cyclosporine A (CSA) group and FZYYG group. The model group was fed with physical salt, while the CSA and FZYYG group were fed with CSA and FZYYG respectively, and then the changes of peripheral hemoglobin (Hb), platelet, bone marrow nucleus cell (BMNC), bone marrow hematopoiesis tissue volume and bcl-2 expression were examined. RESULTS: The count of Hb, the platelet, BMNC, the bone marrow hematopoiesis tissue volume and the bcl-2 positive rate in the model group were lower than those in the normal group (P<0.05), whereas the count of Hb, the platelet, BMNC, the bone marrow hematopoiesis tissue volume and the bcl-2 positive rate in both CAS and FZYYG groups were significantly higher than those in the model group (P<0.05). CONCLUSION: FZYYG can induce the bcl-2 antigen expression and postpone the haematogenous cells apoptosis, and it is effective for mice with immune mediated aplastic anemia.