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Reprogramming of human Peripheral Blood Mononuclear Cell (PBMC) from a Chinese patient suffering Duchenne muscular dystrophy to iPSC line (SDQLCHi007-A) carrying deletion of 49-50 exons in the DMD gene

Duchenne muscular dystrophy (DMD), an X-linked genetic disorder characterized by progressive muscle weakness and atrophies affecting skeletal and cardiac muscles, is caused by mutations in dystrophin (DMD) gene that spans 79 exons. Here, we generated...

Authors: Jingyun Guan, Xinnong Liu, Haiyan Zhang, Xiaomeng Yang, Yanyan Ma, Yue Li, Zhongtao Gai, Yi Liu

SST Peer Reviewed

CX3CR1 Is Expressed in Differentiated Human Ciliated Airway Cells and Co-Localizes With Respiratory Syncytial Virus on Cilia in a G Protein-Dependent Manner

Respiratory syncytial virus (RSV) is the principal cause of bronchiolitis in infants and a significant healthcare problem. The RSV Glycoprotein (G) mediates attachment of the virus to the cell membrane, which facilitates interaction of the RSV Fusion...

Authors: Kwang-Il Jeong 1 , Peter A Piepenhagen 2...

SST Peer Reviewed

A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury

We aimed to assess the neuroprotective effect of a pyruvate dehydrogenase kinase (PDK) inhibitor, Nov3r after ischemia/reperfusion (IR) injury in rats. IR injury was induced by applying...

Authors: Sato K, Mochida S, Tomimoto D, Konuma T, Kiyota N, Tsuda S, Shiga Y, Omodaka K, Nakazawa T

An integration-free iPSC line (SDQLCHi017-A) derived from a patient with nemaline myopathy-2 disease carrying compound heterozygote mutations in NEB gene

Nemaline myopathy-2 (NEM2) is an autosomal recessive skeletal muscle disorder caused by mutations in the nebulin (NEB) gene. We report the generation and characterization of a human induced pluripotent stem cell (iPSC) line SDQLCHi017-A, derived from...

Authors: Yanyan Ma, Haiyan Zhang, Xiaomei Li, Xiaomeng Yang, Yue Li, Jingyun Guan, Yuqiang Lv, Zhongtao...

SST Peer Reviewed

Phenotypic manifestation of a-synuclein strains derived from Parkinson's disease and multiple system atrophy in human dopaminergic neurons

a-Synuclein is critical in the pathogenesis of Parkinson’s disease and related disorders, yet it remains unclear how its aggregation causes degeneration of human dopaminergic neurons. In this study, we induced a-synuclein aggregation in human...

Authors: Tanudjojo B, Shaikh SS, Fenyi A, Bousset L, Agarwal D, Marsh J, Zois C, Heman-Ackah S, Fischer R,...

Olinciguat, an Oral sGC Stimulator, Exhibits Diverse Pharmacology Across Preclinical Models of Cardiovascular, Metabolic, Renal, and Inflammatory Disease

Nitric oxide (NO)-soluble guanylate cyclase (sGC)-cyclic 3',5' GMP (cGMP) signaling plays a central role in regulation of diverse processes including smooth muscle relaxation, inflammation, and fibrosis. sGC is activated by the...

Authors: Zimmer DP, Shea CM, Tobin JV, Tchernychev B, Germano P, Sykes K, Banijamali AR, Jacobson S, Bernier...

Establishment of induced pluripotent stem cell line CSUASOi004-A by reprogramming peripheral blood mononuclear cells of a PRPF6-related dominant retinitis pigmentosa patient

We have established the patient-specific induced pluripotent stem (iPS) cell line CSUASOi004-A by using peripheral blood mononuclear cells (PBMCs) of a retinitis pigmentosa (RP) patient with a PRPF6 gene mutation (c.G2699A:p.R900H). CSUASOi004-A was...

Authors: Yalan Zhou, Yutong Jing, Shengru Mao, Jian Liu, Zekai Cui, Yini Wang, Juan Chen, Hon Fai Chan, Shibo...

CRISPR-Cas9-Mediated Gene Editing in Mouse Spermatogonial Stem Cells

Precise genome editing is a powerful tool for analysis of gene function. However, in spermatogonial stem cells (SSCs), this still remains a big challenge mainly due to low efficiency and complexity of currently available gene editing techniques. The...

Authors: Wang Y, Ding Y, Li J

Interference with ERK-dimerization at thenucleocytosolic interface targets pathologicalERK1/2 signaling without cardiotoxic side-effects

Dysregulation of extracellular signal-regulated kinases (ERK1/2) is linked to several diseases including heart failure, genetic syndromes and cancer. Inhibition of ERK1/2, however, can cause severe cardiac side-effects, precluding its wide...

Authors: Tomasovic A, Brand T, Schanbacher C, Kramer S, Hümmert MW, Godoy P, Schmidt-Heck W, Nordbeck...

A 3D Human Liver Model of Nonalcoholic Steatohepatitis

Background and Aims: To better understand nonalcoholicsteatohepatitis (NASH) disease progression and to evaluatedrug targets and compound activity, we undertook the development of an in vitro 3D model to mimic liver architecture andthe NASH...

Authors: Marion Duriez , Agnes Jacquet , Lucile Hoet , Sandrine Roche , Marie-Dominique Bock , Corinne Rocher...

SST Peer Reviewed

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Reprogramming of human Peripheral Blood Mononuclear Cell (PBMC) from a Chinese patient suffering Duchenne muscular dystrophy to iPSC line (SDQLCHi007-A) carrying deletion of 49-50 exons in the DMD gene

CX3CR1 Is Expressed in Differentiated Human Ciliated Airway Cells and Co-Localizes With Respiratory Syncytial Virus on Cilia in a G Protein-Dependent Manner

A pyruvate dehydrogenase kinase inhibitor prevents retinal cell death and improves energy metabolism in rat retinas after ischemia/reperfusion injury

An integration-free iPSC line (SDQLCHi017-A) derived from a patient with nemaline myopathy-2 disease carrying compound heterozygote mutations in NEB gene

Phenotypic manifestation of a-synuclein strains derived from Parkinson's disease and multiple system atrophy in human dopaminergic neurons

Olinciguat, an Oral sGC Stimulator, Exhibits Diverse Pharmacology Across Preclinical Models of Cardiovascular, Metabolic, Renal, and Inflammatory Disease

Establishment of induced pluripotent stem cell line CSUASOi004-A by reprogramming peripheral blood mononuclear cells of a PRPF6-related dominant retinitis pigmentosa patient

CRISPR-Cas9-Mediated Gene Editing in Mouse Spermatogonial Stem Cells

Interference with ERK-dimerization at thenucleocytosolic interface targets pathologicalERK1/2 signaling without cardiotoxic side-effects

A 3D Human Liver Model of Nonalcoholic Steatohepatitis