Products

Online Inquiry

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

  • Verification code
    Click image to refresh the verification code.

Ocular System


Vision loss can be caused by multiple complications which involve one or more ocular degenerative diseases, such as age-related macular degeneration (AMD), retinitis pigmentosa (RP), and glaucoma, which is the leading cause of irreversible vision loss in developed countries. According to recorded prevalence and UN World Population Prospect data, AMD, the leading cause of sight loss in developed countries will affecting 196 million people worldwide in 2020.

Damage or degeneration of any part of the retina is permanent and irreversible, pathological molecular/cellular mechanism isn’t fully clear to date. The major barrier may be lacking access to in vitro models that can reflect the natural progression and phenotype of ocular degenerative diseases, to explore molecular pathological mechanism and discover new drug and methods. 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, and share the self-renewal and pluripotency characteristics that can be induced to differentiate into multiple types of retinal cells. The emerging technology of disease-specific iPSC-derived disease models bridge the gap between clinical phenotype and molecular/cellular mechanisms, facilitating new strategies for drug screening, and developing novel therapeutic agents for clinical trials. An increasing number of researches have explored the utility of iPSCs to model a variety of degenerative diseases affecting different retinal cell types, including retinal ganglion cells (RGCs), retinal pigmented epithelium (RPE) and photoreceptors. Creative Biolabs provides a series of patient-specific iPSC models of ocular degenerative diseases for different research demands of worldwide clients.

Drug discovery pipeline using iPSC. Fig 1. Drug discovery pipeline using iPSC.

By combination with other promising technologies in tissue engineering and gene edition, iPSC-derived ocular disease models have huge potential in exploring ocular degenerative disease’s pathological mechanisms, as well as application in drug discovery/screening, toxicology testing and estimating regenerative medicine/methods.

The Generation Steps of hPSC-based Disease Models

Retinal Cell Lineages Differentiation of hPSCs

Differentiation of human pluripotent stem cells (hPSCs) into the retinal cell lineages, including retinal ganglion cells (RGCs), retinal pigmented epithelium (RPE) and photoreceptors, is a multistep process, during which multipotent retinal progenitors produce and then generate RGCs, RPE and photoreceptors by different pathways of differentiation.

Schematic of effect of select signaling molecules on specific stages of PSC photoreceptor differentiation in vitro. Fig 2. Schematic of effect of select signaling molecules on specific stages of PSC photoreceptor differentiation in vitro.

Features of STEMODTM Ocular Disease Models

hPSC Models of Ocular Diseases

Human PSC-retinal cell (hPSC-RC) models of inherited ocular degenerative diseases are used to investigate the underlying disease mechanisms and evaluate therapeutic options in a patient-specific manner. Creative Biolabs offers a state-of-the-art STEMODTM ocular disease models platform which provides a list of hPSC-RC models with accurate evaluated retinal cell phenotypes. These ocular degenerative disease models are prepared for various disease types including POGA, BEST disease, AMD and retinitis pigmentosa. If the desired disease models for your research are not found in the product list, please contact us and the customized service can also be provided for you!

References

  1. Flaxman, S.; et al. (2017). Global causes of blindness and distance vision impairment 1990-2020: a systematic review and meta-analysis. The Lancet Global Health, 5(12), pp. e1221-e1234.
  2. Klettner, A. (2015). Age-related macular degeneration-biology and treatment. Medizinische Monatsschrift fur Pharmazeuten, 38(7), 258-64.
  3. Park, S.J.; et al. (2014). Age-related macular degeneration: prevalence and risk factors from Korean National Health and Nutrition Examination Survey, 2008 through 2011. Ophthalmology, 121(9), 1756-1765.
  4. Song, M.J.; Bharti, K. (2016). Looking into the future: Using induced pluripotent stem cells to build two- and three-dimensional ocular tissue for cell therapy and disease modeling. Brain Research, 1638, 2-14.

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

Datasheet INQUIRY
Target Gene: TBK1
Mutation: Duplicate on chromosome 12q14
Organ System: Ocular System
Disease: Primary Open Angle Glaucoma (POAG)
Datasheet INQUIRY
Target Gene: OPTN
Mutation: E50K
Organ System: Ocular System
Disease: Primary Open Angle Glaucoma (POAG)
Datasheet INQUIRY
Target Gene: ARMS2
Mutation: g.5270G> T (p.Ala69Ser)
Organ System: Ocular System
Disease: Age-Related Macular Degeneration (AMD)
Datasheet INQUIRY
Target Gene: HTRA1
Mutation: 4504G> A
Organ System: Ocular System
Disease: Age-Related Macular Degeneration (AMD)
Datasheet INQUIRY
Target Gene: BEST1
Mutation: A146K
Organ System: Ocular System
Disease: Bestrophinopathy
Datasheet INQUIRY
Target Gene: BEST1
Mutation: N296H (1591A> C)
Organ System: Ocular System
Disease: Bestrophinopathy
Datasheet INQUIRY
Target Gene: BEST1
Mutation: R218H
Organ System: Ocular System
Disease: Bestrophinopathy
Datasheet INQUIRY
Target Gene: BEST1
Mutation: L234P
Organ System: Ocular System
Disease: Bestrophinopathy
Datasheet INQUIRY
Target Gene: BEST1
Mutation: A243T
Organ System: Ocular System
Disease: Bestrophinopathy
Datasheet INQUIRY
Target Gene: RP1
Mutation: 721Lfs722X (c.2161 2162insC)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: PRPH2
Mutation: W316G (c.946T> G)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: RHO
Mutation: G188R (c.562G> A)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: RP9
Mutation: H137L (c.401A> T)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: RHO
Mutation: E181K (541G> A)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: RP2
Mutation: R120X (c.519C> T)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: MFRP
Mutation: IVS10 + 5G> A (243G> A)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: MFRP
Mutation: 492Cdel
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)
Datasheet INQUIRY
Target Gene: MERTK
Mutation: c.992_993delCA (p.Ser331Cysfs*5)
Organ System: Ocular System
Disease: Retinitis Pigmentosa (RP)

Interested in our expertise?

Contact us for more information

Get free consultations

USA

  • 45-1 Ramsey Road, Shirley, NY 11967, USA
  • Tel: 1-631-381-2994
  • Fax: 1-631-207-8356
  • Email:

Europe

Easy access to products and services you need from our library via powerful searching tools

Enter your email here to subscribe.

Submit

Follow us on

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

Copyright © 2007 - 2021 Creative Biolabs. All Rights Reserved.