Angelman Syndrome [AG1-0] Human Induced Pluripotent Stem Cells

These induced pluripotent stem cells (iPSCs) have been derived from previously banked fibroblasts from a female individual with Angelman syndrome (AS). Like the person they were derived from, these iPSCs have an approximately 5 Mb deletion of maternal 15q11.2-q13.1.

Angelman syndrome (AS) is a neurogenetic disorder that leads to severe mental retardation, lack of speech, seizures, and ataxia. AS is caused by loss of function of the maternally inherited allele of UBE3A. The UBE3A gene encodes an E3 ubiquitin ligase and is subject to tissue-specific genomic imprinting; it is expressed exclusively from the maternal allele in neurons, but is bi-allelically expressed in other tissues. Approximately 70% of individuals with AS have a large deletion of the maternal allele of chromosome 15q11-q13.

From the laboratories of Stormy J. Chamberlain, PhD and Marc Lalande, PhD, University of Connecticut.

The Investigator's Annexe Part of The Investigator's Annexe program.

Catalog Number Product Size AVAILABILITY Price Qty
ECN001
Angelman Syndrome [AG1-0] Human iPSCs, live cells in T25 flask
1 vial 2-3 weeks
Price: $366.00

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Specifications
Name: AG1-0
Cell Type: Induced pluripotent stem cell (iPSC)
Organism: Human
Accession ID: CVCL_U498
Morphology: Small round cells that grow in colonies, indistinguishable from human embryonic stem cells
Biosafety Level: 2
Subculturing: Manually split no more than 1:5, depending on density of dish
Growth Conditions: hESC medium (DMEM/F12 with 20% Knockout serum replacer, 1X Non-essential amino acids, 1mM L-glutamine, 4ug/mL basic Fibroblast growth factor)
Cryopreservation: 60% DMEM/F12, 30% FBS, 10% DMSO
Source: Fibroblasts
Storage: Cells should be cultured at 37C upon arrival. Cryopreserved cells should be stored in Liquid nitrogen
Shipped: Ambient temperature as live cells in T25 flask
Provider
From the laboratories of Stormy J. Chamberlain, PhD and Marc Lalande, PhD, University of Connecticut.
Comments

These cells have been propagated using irradiated mouse embryonic fibroblasts as a feeder layer. It is recommended to keep the cells on a feeder layer until established.

References
  1. Sun AX, Yuan Q, Tan S, Xiao Y, Wang D, Khoo AT, Sani L, Tran HD, Kim P, Chiew YS, Lee KJ, Yen YC, Ng HH, Lim B, Je HS. Direct Induction and FunctionalMaturation of Forebrain GABAergic Neurons from Human Pluripotent Stem Cells. CellRep. 2016 Aug 16;16(7):1942-53. View Article
  2. Chamberlain SJ, Chen PF, Ng KY, Bourgois-Rocha F, Lemtiri-Chlieh F, Levine ES, Lalande M. Induced pluripotent stem cell models of the genomic imprinting disorders Angelman and Prader-Willi syndromes. Proc Natl Acad Sci U S A. 2010 Oct 12;107(41):17668-73.

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