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#34118793   2021/06/12 To Up

Fetal double outlet right ventricle without heterotaxy syndrome: diagnostic spectrum, associated extracardiac pathology and clinical outcomes.

To document the clinical spectrum and outcomes of fetal DORV without heterotaxy in a recent diagnostic era.
Aisling A Young, Angela McBrien, Oana Caluseriu, Kim Haberer, Gayathri Wewala, Luke Eckersley, Lisa K Hornberger

2970 related Products with: Fetal double outlet right ventricle without heterotaxy syndrome: diagnostic spectrum, associated extracardiac pathology and clinical outcomes.



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#34118792   2021/06/11 To Up

Physiological and pathological functions of βB2-crystallins in multiple organs: a systematic review.

Crystallins, the major constituent proteins of mammalian lenses, are significant not only for the maintenance of eye lens stability, transparency, and refraction, but also fulfill various physiopathological functions in extraocular tissues. βB2-crystallin, for example, is a multifunctional protein expressed in the human retina, brain, testis, ovary, and multiple tumors. Mutations in the βB2 crystallin gene or denaturation of βB2-crystallin protein are associated with cataracts, ocular pathologies, and psychiatric disorders. A prominent role for βB2-crystallins in axonal growth and regeneration, as well as in dendritic outgrowth, has been demonstrated after optic nerve injury. Studies in βB2-crystallin-null mice revealed morphological and functional abnormalities in testis and ovaries, indicating βB2-crystallin contributes to male and female fertility in mice. Interestingly, although pathogenic significance remains obscure, several studies identified a clear correlation between βB2 crystallin expression and the prognosis of patients with breast cancer, colorectal cancer, prostate cancer, renal cell carcinoma, and glioblastoma in the African American population. This review summarizes the physiological and pathological functions of βB2-crystallin in the eye and other organs and tissues and discusses findings related to the expression and potential role of βB2-crystallin in tumors.
Meihui Li, Shengnan Liu, Wei Huang, Junjie Zhang

2796 related Products with: Physiological and pathological functions of βB2-crystallins in multiple organs: a systematic review.



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#34118692   2021/06/09 To Up

High throughput proteomic and metabolic profiling identified target correction of metabolic abnormalities as a novel therapeutic approach in head and neck paraganglioma.

Head and neck paragangliomas (HNPGLs) are rare neoplasms that represent difficult treatment paradigms in neurotology. Germline mutations in genes encoding succinate dehydrogenase (SDH) are the cause of nearly all familial HNPGLs. However, the molecular mechanisms underlying tumorigenesis remain unclear. Mutational analysis identified 6 out of 14 HNPGLs harboring clinicopathologic SDH gene mutations. The SDHB gene was most frequently mutated in these patients, and western blot showed loss of SDHB protein in tumors with SDHB mutations. The paraganglioma cell line (PGL-626) was established from a sample that harbored a missense SDHB mutation (c.649C > T). Spectrometric analysis using tandem mass tags identified 151 proteins significantly differentially expressed in HNPGLs compared with normal nerves. Bioinformatics analyses confirmed the high level of enrichment of oxidative phosphorylation and metabolism pathways in HNPGLs. The mitochondrial complex subunits NDUFA2, NDUFA10, and NDUFA4, showed the most significantly increased expression and were localized predominantly in the cytoplasm of PGL-626 cells. The mitochondrial complex I inhibitor metformin exerted dose-dependent inhibitory effects on PGL-626 cells via cooperative down-regulation of NDUFA2, 4, and 10, with a significant decrease in the levels of reactive oxygen species and mitochondrial membrane potential. Further metabolomic analysis of PGL-626 cells showed that metabolites involved in central carbon metabolism in cancer and sphingolipid signaling pathways, pantothenate and CoA biosynthesis, and tryptophan and carbon metabolism were significantly altered after metformin treatment. Thus, this study provides insights into the molecular mechanisms underlying HNPGL tumorigenesis and identifies target correction of metabolic abnormalities as a novel therapeutic approach for this disease.
Zhigang Wang, Hongsai Chen, Lu Xue, Weiwei He, Wenying Shu, Hao Wu, Zhaoyan Wang

1062 related Products with: High throughput proteomic and metabolic profiling identified target correction of metabolic abnormalities as a novel therapeutic approach in head and neck paraganglioma.

0.1ml (1mg/ml)400Tests

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#34118585   2021/05/28 To Up

The occurrence of myelin oligodendrocyte glycoprotein antibodies in aquaporin-4-antibody seronegative Neuromyelitis Optica Spectrum Disorder: A systematic review and meta-analysis.

Despite inclusion in neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated diseases are increasingly recognized as an independent disease entity. In this study, we conducted a systematic review and meta-analysis to comprehensively update the rate of occurrence of MOG-Ab in Aquaporin4 (AQP4)-antibody seronegative NMOSD.
Xindi Li, Chengyi Zhang, Dongmei Jia, Moli Fan, Ting Li, De-Cai Tian, Yaou Liu, Fu-Dong Shi

1521 related Products with: The occurrence of myelin oligodendrocyte glycoprotein antibodies in aquaporin-4-antibody seronegative Neuromyelitis Optica Spectrum Disorder: A systematic review and meta-analysis.

4 Arrays/Slide4 Membranes/Box4 Membranes/Box4 Membranes/Box4 Arrays/Slide2 Pieces/Box4 Membranes/Box4 Membranes/Box4 Arrays/Slide4 Arrays/Slide4 Membranes/Box

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#34118459   2021/06/10 To Up

Inflammatory bowel disease in primary immunodeficiency disorders is a heterogeneous clinical entity requiring an individualized treatment strategy: A systematic review.

To describe the prevalence, clinical presentation and current treatment regimens of inflammatory bowel disease (IBD) in patients with primary immunodeficiency disorders (PIDs).
Tim Both, Virgil A S H Dalm, Savannah A Richardson, Naïma van Schie, Luuk M van den Broek, Annemarie C de Vries, P Martin van Hagen, Saskia M Rombach

1205 related Products with: Inflammatory bowel disease in primary immunodeficiency disorders is a heterogeneous clinical entity requiring an individualized treatment strategy: A systematic review.

100 100ug Lyophilized100 ul100ug Lyophilized100ug Lyophilized

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#34118354   2021/06/09 To Up

Glucose metabolic crosstalk and regulation in brain function and diseases.

Brain glucose metabolism, including glycolysis, the pentose phosphate pathway, and glycogen turnover, produces ATP for energetic support and provides the precursors for the synthesis of biological macromolecules. Although glucose metabolism in neurons and astrocytes has been extensively studied, the glucose metabolism of microglia and oligodendrocytes, and their interactions with neurons and astrocytes, remain critical to understand brain function. Brain regions with heterogeneous cell composition and cell-type-specific profiles of glucose metabolism suggest that metabolic networks within the brain are complex. Signal transduction proteins including those in the Wnt, GSK-3β, PI3K-AKT, and AMPK pathways are involved in regulating these networks. Additionally, glycolytic enzymes and metabolites, such as hexokinase 2, acetyl-CoA, and enolase 2, are implicated in the modulation of cellular function, microglial activation, glycation, and acetylation of biomolecules. Given these extensive networks, glucose metabolism dysfunction in the whole brain or specific cell types is strongly associated with neurologic pathology including ischemic brain injury and neurodegenerative disorders. This review characterizes the glucose metabolism networks of the brain based on molecular signaling and cellular and regional interactions, and elucidates glucose metabolism-based mechanisms of neurological diseases and therapeutic approaches that ameliorate metabolic abnormalities in those diseases.
Shuai Zhang, Brittany Bolduc Lachance, Mark P Mattson, Xiaofeng Jia

1839 related Products with: Glucose metabolic crosstalk and regulation in brain function and diseases.

1 g200 96 wells (1 kit)500 tests200ul1000 TESTS/0.65ml10 mg2 Pieces/Box100ug100 mg

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#34118271   2021/06/09 To Up

"I'm not going to be a guinea pig:" medical mistrust as a barrier to male contraception for Black American men in Los Angeles, CA.

Racial disparities in unintended pregnancy and contraceptive use in the United States are not mediated by access to family planning services alone. Rather, a history of medical mistrust underlies Black Americans' adoption of new medical technologies, inclusive of contraception. Efforts to develop hormonal male contraceptives need to incorporate Black Americans' experiences and perspectives so that new contraceptives enable their reproductive goals and promote gender equity.
Brian T Nguyen, Anthony L Brown, Felica Jones, Loretta Jones, Mellissa Withers, Katharine M Ciesielski, Jennifer M Franks, Christina Wang

1076 related Products with: "I'm not going to be a guinea pig:" medical mistrust as a barrier to male contraception for Black American men in Los Angeles, CA.

1 kit1 kit1 kit(96 Wells)1,000 tests1 kit(96 Wells)1 kit(96 Wells)200 1 kit(96 Wells)1 kit(96 Wells)

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