Background Congenital fibrosis of extraocular muscles (CFEOM) affects patient' s appearance and quality of life,and no effective treatment for this disease is available.Imaging study is helpful for exploring the pathogenesis of CFEOM.Objective This study was to describe the characteristics of CFEOM associated with limb movement disorder using magnetic resonance imaging (MRI).Methods A family with CFEOM associated with limb movement disorder was investigated in Renmin Hospital of Wuhan University.Disease history was collected and the pedigree was investigated.Ophthalmologic examinations,including corrected visual acuity,refractive error,slitlamp examination,ophthalmoscopic examination,force of levator palpebrae superioris,ocular movement,eye position,forced duction test,and bell phenomenon examination,were performed.Ocular orbital and cranial MRI was performed in 4 CFEOM patients and 10 normal subjects to compare the structures of the extraocular muscles,motor nerve and cranium.Oral informed consent was obtained from each patient prior to any medical examination.Results A total of 1 1 members from 3 generations were investigated in this study,presenting with 4 cases of disease.The mode of inheritance of this family complied with the Mendelian autosomal dominant inheritance law.Clinical signs included disturbance of eye movement,deviation of eye position,ptosis,lack of Bell sign and positive reaction of passive pull test.In addition,unstable gait,improper body limb alignment,dysphasia and mental retardation were ohserved in 1 patient,which coincided with the diagnostic criteria of type 3 CFEOM.MRI results demonstrated that the levator palpebrae superioris,superior rectus and superior oblique muscle were clearly thinner,and the medial rectus,lateral rectus,inferior rectus muscle were thinning in the patients,showing significant differences in comparison with the normal controls(P＜O.05).The oculomotor and abducens nerves became thinner and even absent in the patients.Cranial MRI showed that Ⅲ-3 in the pedigree with callosum was shorter than that of the normal controls,suggesting that patient suffered from corpus callosum hypoplasia.Meanwhile,cranial MRI indicated the presence of cerebellar hypoplasia and the expansion of the fourth ventricle.Conclusions MRI demonstrates consistent abnormalities of the oculomotor nerves and abducens nerves in the affected individuals in this CFEOM 3 family,and some affected members exhibit two types of central nervous system abnormalities-corpus callosum and cerebellar hypoplasia.These findings suggest that CFEOM 3 is primarily a neuronal disease.

Objective To explore the anatomical characteristic of the third palmar interosseous mus- cle as well its dominate nerve,and to investigate the anatomical basis of difficult recovery of digitus minimus adduction.Methods Twenty aduh fresh hands without deformity and trauma were obtained.Dissect and observe the third palmar interosseous muscle and its dominate nerve and adjacent structure under surgical mi- croseope,measure the size of the third pahnar interosseous muscle and its dominate nerve,the data were pro- cessed by stastistics method.Results Among palmar interosseous muscles and its dominate nerves,the third palmar interosseous muscle and its dominate nerve is the smallest.There are conspicuous tendon bundle on the surface of the third palmar interosseous muscle partly,which have a potential compression on the third palmar interosseous muscle dominting nerve.Conclusion The third palmar interosseous muscle is the smal- lest among palmar interusseous muscles and it is the only digitus minimus adduction muscle.The sominating nerve of the third palmar interosseous muscle is small anti the tendon bundle of the third palmar interosseous muscle have a potential compression.All these can cast light on diffcult recovery of digitus minimus adduction.

This study investigated the effect of puerarin on human umbilical vein endothelial cells (HUVEC) injured with hydrogen peroxide (H₂O₂). HUVEC were divided into three groups: a control group, a model group (H₂O₂ 400 μmol·L^-1) and a puerarin-treated group (3, 10, 30 and 100 μmol·L^-1). HUVEC were cultured with varied concentration of puerarin for 2 h and treated with H₂O₂ for another 24 h. Cell proliferation was detected by a CCK-8 assay. The mitochondrial membrane potential was measured by a JC-1 fluorescent probe. A transwell chamber assay was adopted to observe cell migration ability. Mitochondrial respiratory function was measured in a two-chamber titration injection respirometer (Oxygraph-2k). The expression of interleukin-1β (IL-1β), interleukin-18 (IL-18) and tumor necrosis factor-α (TNF-α) was detected by quantitative real-time PCR. The expression of pyroptosis-mediated proteins, including cleaved-cysteinyl aspartate-specific proteinase-1 (caspase-1), N-gasdermin D (N-GSDMD), NOD-like receptor protein 3 (NLRP3) and purinergic ligand-gated ion channel 7 receptor (P2X7R) was detected by Western blot. The results show that 400 μmol·L^-1 H₂O₂ treatment for 24 h causes obvious damage to HUVEC. Compared with the model group, puerarin protected against cellular injury in a dose-dependent manner, with the greatest effect at a dose of 30 and 100 μmol·L^-1. Puerarin significantly decreased the mitochondrial membrane potential and improved mitochondrial function. Puerarin inhibited cell migration induced by H₂O₂, suppressed the expression of IL-1β, IL-18 and TNF-α, and down-regulated the pyroptosis-mediated protein. These changes are statistically significant (P < 0.05). These findings demonstrate that puerarin has a protective effect against H₂O₂-induced oxidative damage of HUVEC by inhibiting the migration of HUVEC cells. The mechanism may be related to improved mitochondrial respiratory function and inhibition of pyroptosis.

AIMTo investigate the spatial and temporal expression of germ cell nuclear factor (GCNF) in mouse and rat epididymis during postnatal period.

METHODSThe epididymal sections from different postnatal days were stained for GCNF by the indirect immunofluorescence technique and digital photographs were taken by a Carl Zeiss confocal microscope.

RESULTSGCNF was first detected on day 12 in mouse epididymis and day 14 in rat epididymis. The highest expression of GCNF was observed on day 35 in both mouse and rat epididymis. In adults, GCNF exhibited a region-specific expression pattern, i.e., it was expressed predominantly in the initial segment, caput and proximal corpus of rat epididymis and was abundant in the proximal corpus of mouse epididymis. GCNF could be found in the nuclei of the principal, apical, narrow, clear and halo cells.

CONCLUSIONGCNF may play an important role in epididymal differentiation and development and in sperm maturation.

Aging ; Animals ; DNA-Binding Proteins ; analysis ; Epididymis ; chemistry ; growth & development ; Fluorescent Antibody Technique, Indirect ; Male ; Mice ; Mice, Inbred BALB C ; Microscopy, Confocal ; Nuclear Receptor Subfamily 6, Group A, Member 1 ; Rats ; Rats, Sprague-Dawley ; Receptors, Cytoplasmic and Nuclear ; analysis ; Tissue Distribution

ATP-Binding Cassette, Sub-Family B, Member 1 ; analysis ; Cytotoxicity, Immunologic ; Dendritic Cells ; physiology ; Drug Resistance, Multiple ; Drug Resistance, Neoplasm ; Humans ; Immunophenotyping ; K562 Cells ; Leukemia ; drug therapy ; immunology ; Lymphocyte Culture Test, Mixed

0.05).In group Ⅱand Ⅲ,the carotid artery IMT was thicker and the amount of plagues were larger than those in group Ⅰ(P

This study aimed to evaluate the vasorelaxant effect and mechanisms of compound reserpine and triamterene tablets (CRTTs) and its component triamterene on isolated rat thoracic aorta rings. Isolated rat thoracic aorta rings pre-contracted by high potassium or norepinephrine (NE) were used to evaluate the vasodilatory effect of CRTTs and its component triamterene. The mechanisms concerning endothelium, potassium channels and calcium channels were studied through the interventions of several tool drugs. Animal welfare and experimental procedures followed the requirements of the Laboratory Animal Management and Animal Welfare Ethics Committee of the Institute of Materia Medica, Chinese Academy of Medical Sciences and Peking Union Medical College. The results showed that both CRTTs and triamterene had potent relaxant effect on KCl and NE pre-contracted vessels. Triamterene showed partial endothelium dependency, and N-nitro-L-argininemethyl ester hydrochloride (L-NAME) could influence the relaxant effect, which may be related to the opening of calcium-activated potassium channels. Meanwhile, CRTTs could inhibit intracellular Ca²⁺ release and extracellular Ca²⁺ influx. Overall, CRTTs and its component triamterene have vasorelaxant effects on vessels in vitro, and the vasorelaxant effect of CRTTs may be related to intracellular Ca²⁺ release and extracellular Ca²⁺ influx, while this effect of triamterene may depend on vascular endothelial function and may also be related to the opening of calcium-activated potassium channels.

Objective To explore the intervention effect and mechanism of sitagliptin on renal injury in type 1 diabetic (T1DM) mice.Methods The modeling group was intraperitoneally injected with streptozotocin (150 mg· kg-1) once and blood glucose ≥ 16.7 mmol · L-1 was considered as diabetes mellitus (DM).DM mice were randomly divided into two groups:model group and experimental group.Experimental group was treated with sitagliptin (15 mg · kg-1 · d-1) via intragastric administration for 4 weeks while the mice in model group and normal group treated with equal volume of ultrapure water.The 24 h microalbuminuria(ALB) was determined after administration.At the end of the experiment,urea nitrogen (BUN) and creatinine (SCr) in serum was detected.The protein expression levels of transforming growth factor-β1 (TGF-β1),Smad2,Smad3 were measured by Western blot.Results After administration sitagliptin for 4 weeks,the contents of ALB in model group and experimental group were (11.96 ± 3.36) (2.46 ±0.97) mg/24 h with significantly(P ＜0.001).The index of kidney weight/body weight(KW/BW) in normal group,model group and experimental group were 15.20 ±2.24,21.43 ±2.16,15.14 ±4.14;compared with normal group,the difference was significantly increased (P ＜ 0.05);compared with model group,the difference was significantly increased (P ＜0.05).The SCr in model group and experimental group were (352.58 ±47.09),(238.51 ± 53.03) μmol · L-1;the BUN in the two groups were(26.08 ±4.65),(10.40 ±2.47)mmol · L-1;compared with model group,the difference was significantly increased (P ＜0.01,P ＜0.001).The expression levels of TGF-β1,Smad2/3 and p-Smad2/3 in normal group,model group and experimental group were 0.19 ±0.02,0.12 ±0.02,0.07 ±0.01;0.23 ±0.02,0.27 ±0.04,0.13 ±0.01;0.18 ±0.01,0.14 ±0.01,0.11 ±0.00,compared with normal group,the difference was significantly increased (P＜0.05,P＜0.01);compared with model group,the difference was significantly increased (P ＜0.05,P ＜0.01).Conclusion Experimental delaying the progression of T1DM nephropathy without not decreasing blood glucose can effect partly through inhibiting TGF-β1/Smad2/3 pathway.

OBJECTIVETo investigate the effects of ultrasound mediated microbubble destruction on capillary permeability in rat skeletal muscles.

METHODSEighteen SD rats were randomized into 3 groups, namely the Evans blue (EB) group, EB+ultrasound (E+U) group and EB+microbubble+ultrasound (U+E+M) group with corresponding treatments, using EB injected into the carotid artery as the indicator for capillary permeability. The microbubbles were injected through the carotid artery with fixed ultrasound parameters. The spillover of EB was estimated under fluorescence microscope according to the visual staining scores. The contents of EB in the skeletal muscles were calculated according to the standard curve and spectrophotometry.

RESULTSEB spillover was observed around the capillaries in E+U+M group, which had a significantly higher visual score than EB group and E+U group (0 and 0-1, respectively, P<0.05). The EB content was 51.57-/+3.89 microg/g in E+U+M group, also significantly higher than those in EB group (28.99-/+4.67 microg/g) and E+U group (30.99-/+4.11 microg/g) (P<0.05).

CONCLUSIONExposure to both ultrasound and microbubble contrast agents results in increased capillary permeability of rat skeletal muscles, which might be an important mechanisms for gene delivery enhancement by ultrasound contrast agents.

Animals ; Capillary Permeability ; physiology ; Coloring Agents ; administration & dosage ; pharmacokinetics ; Contrast Media ; administration & dosage ; Evans Blue ; administration & dosage ; pharmacokinetics ; Female ; Male ; Microbubbles ; Microscopy, Fluorescence ; Muscle, Skeletal ; blood supply ; metabolism ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Spectrophotometry ; Ultrasonics

Objective To investigate the prevalence of dyslipidemia and borderline dyslipidemia and other coronary heart disease(CHD)risk factors in residents from Beijing communities.Methods Resident samples were selected brv random cluster multistage method.Serum hpid level and CHD factors were obtained through questionnaire,physical examination and biocbemical analysis during June 2007 to August 2007 from 10 054 residents.Results Age-and sex-adjusted standardized prevalence of hypercholesterolemia,high low-density lipoprotein cholesterolemia,low high-density lipoprotein cholesterolemia,and triglyceridemia were 9.3%,2.56%,18.79%and 16.84%,respectively.Incidence of borderline hypercholesterolemia was 23.96%.Incidence of dyslipidemia and borderline dyslipidemia was 31.23%and 23.30%respectively,71.17%residents have at least 1 CHD major risk factor and 20.23%residents were in borderline CHD risks.Prevalence of hypertension,diabetes,smoking and obese was interval)in residents having 1 borderline CHD risk factor(RF),2 RF,and 3 RF versus none RF was 1.668,(95%CI:1.319-2.110),2.537(95%CI:1.989-3.235),and 3.203(95%CI:2.007-5.114).Condusions The prevalence of dyslipidemia of residents from Beijing communities is higher the average level of China(25.1%).Over 1 out of 5 residents were in increased risk for borderline dyslipidemia or CHD risks.Intensive control of dvslipidemia and CHD risk factors aro warranted in Beijing residents.