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Cardiovascular System


Cardiovascular disease (CVD), also called as the heart and circulatory disease, is a class of diseases involving the heart or blood vessels that are the leading cause of death globally. The molecular basis research of many cardiac diseases has been hindered by the lack of appropriate in vitro cell culture models that can reflect the phenotype of human disease accurately. Using iPSCs can better understand cardiomyopathies, vascular disorders, rhythm disorders and metabolic risk factors for ischemic heart disease. Pluripotent stem cells (PSCs) have the potential to indefinite self-renewal and differentiation into cells and tissue derivatives from all three germ layers. Induced pluripotent stem cells (iPSCs) are generated by forcing expression of a defined set of transcription factors to reprogram somatic cells into an embryonic pluripotent state. IPSCs can be used in vitro cardiac disease modeling and used for high-throughput pharmacological screenings for drug discovery. Creative Biolabs provides vast patient-specific iPSC models of genetically inherited cardiovascular diseases for the different research demands of worldwide clients.

Combined with other novel technologies in the areas of tissue engineering and gene-targeted manipulation, iPSC cardiovascular disease models have contributed on molecular understanding of pathological mechanisms, early diagnosis, drug discovery, toxicology testing and regenerative medicine. The ability to generate, expand and purify functional cardiac cells is critical to the development of iPSC models of human cardiovascular disease.

The generation steps of hPSC-based disease models

Cardiovascular Lineages Differentiation of hPSCs

Differentiation of human pluripotent stem cells (hPSCs) into the cardiovascular lineages, including cardiomyocytes, vascular endothelial cells and smooth muscle cells, is a multistep process that contains initial epithelial to mesenchymal transition, mesodermal, followed by cardiogenic specification and differentiation, and finally functional maturation. Differentiation of hPSCs is regulated by four major signaling pathways (BMP, FGF, WNT/b-catenin and TGF-b/activin/nodal). During this phase, multipotent cardiovascular progenitors arise and then generate cardiomyocytes, vascular endothelial cells and smooth muscle cells by different pathways of differentiation.

Cardiovascular lineages differentiation of hPSCs Fig 1. Pathways and factors involved in cardiac differentiation of human pluripotent stem cells in vitro

Features of STEMODTM Cardiovascular Disease Models

hPSC Models of Cardiac Diseases

hPSC-cardiomyocyte (hPSC-CM) models of inherited cardiac diseases are used to investigate the underlying disease mechanisms and evaluate therapeutic options in a patient-specific manner. Creative Biolabs has built a state-of-the-art STEMODTM cardiovascular disease models platform which provides a list of hPSC-CM models with accurate evaluated cardiomyocyte phenotypes. These cardiovascular disease models are prepared for the investigation of primary arrhythmias, primary cardiomyopathies and cardiometabolic disorders. If you don’t find the cardiovascular disease models you want in the product list, we also provide the customized service for you!

Reference

  1. Moretti, A., Laugwitz, K. L., Dorn, T., Sinnecker, D., & Mummery, C. (2013). Pluripotent stem cell models of human heart disease. Cold Spring Harbor perspectives in medicine, 3(11), a014027.
  2. Brandão, K. O., Tabel, V. A., Atsma, D. E., Mummery, C. L., & Davis, R. P. (2017). Human pluripotent stem cell models of cardiac disease: from mechanisms to therapies. Disease models & mechanisms, 10(9), 1039-1059.

For Lab Research Use Only, Not for Human or Animal Therapeutic Use.

Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: R190Q (G569A)
Organ System: Cardiovascular System
Disease: LQT1
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: 1893delC (P631fs/33)
Organ System: Cardiovascular System
Disease: LQT1
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: G269S (G805A)
Organ System: Cardiovascular System
Disease: LQT1
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: G345E (G1034A)
Organ System: Cardiovascular System
Disease: LQT1
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: T587M
Organ System: Cardiovascular System
Disease: LQT1
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: c.922~1,032 del, p.308~344 del
Organ System: Cardiovascular System
Disease: LQT1
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: R594Q (c.1781G>A)
Organ System: Cardiovascular System
Disease: LQT1/JLNS
Datasheet INQUIRY
Target Gene: KCNQ1 (KVLQT1, Kv7.1)
Mutation: E160fs (c.478-2A>T)
Organ System: Cardiovascular System
Disease: LQT1/JLNS
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: G1681A (p.561A>T)
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: A561V
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: A614V (C821T)
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: N996I (c.2987A>T)
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: c.2399-28A>G
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: A422T (1264G>A)
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: R176W
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: KCNH2
Mutation: A561P
Organ System: Cardiovascular System
Disease: LQT2
Datasheet INQUIRY
Target Gene: SNC5A
Mutation: p.R1644H
Organ System: Cardiovascular System
Disease: LQT3/BrS
Datasheet INQUIRY
Target Gene: SNC5A
Mutation: F1473C
Organ System: Cardiovascular System
Disease: LQT3/BrS
Datasheet INQUIRY
Target Gene: SNC5A
Mutation: p.V1763M (c.5287G> A)
Organ System: Cardiovascular System
Disease: LQT3/BrS
Datasheet INQUIRY
Target Gene: SNC5A
Mutation: N406K (1218C>A)
Organ System: Cardiovascular System
Disease: LQT3/BrS

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