AFF2 Knock-out Human Induced Pluripotent Stem Cells (Episomal, HFF)
|Catalog#||Unit||Unit Price (USD)||Actions|
|iPS61||5x105 cells/vial||$2,835.00||Add to Cart|
ALSTEM offers human iPS cell line (iPS61) with AF4/FMR2 Family Member 2 (AFF2) deficiency. This cell line is generated from pre-made footprint-free human iPS cells (cat# iPS11) by using gene editing method to introduce a deletion and a frame shift in the AFF2 gene. Sequencing results confirmed that this isogenic iPSC line has a stable homozygous 94 bp deletion in the exon 8 of AFF2 gene. When cultured under standard human ES cell culture conditions, the morphology of human iPS cells is identical to that of human ES cells. Potentially, this cell line can be a good model for studying AD-related pathologies in neurons induced specifically by AFF2.
- Good model for studying ALS-related pathologies in neurons associated with AFF2
- Ideal for drug discovery when used with AFF2 wild type iPS cell line (cat# iPS11)
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two devastating neurodegenerative disorders lacking effective treatments. ALS, known as Lou Gehrig’s disease, causes progressive muscle weakness and atrophy. FTD is characterized by the progressive decline of cognition, behavior changes, and/or language impairment. Previous studies demonstrated that one of the most common genetic causes of ALS and FTD is the expansion of GGG GCC (G4C2) repeat in the first intron of C9ORF72 which leads to partial loss of C9ORF72 function. A recent genetic study discovered Lilli, the only Drosophila homolog of human AFF2 gene, as a strong transcriptional suppressor of GC-rich sequences. Further studies argued that knockout of AFF2 in iPS cells reduced the disease-causing proteins generated by GC-rich repeats and rescued disease phenotypes in human neurons.
Alstem’s footprint-free human iPS cell line (cat# iPS61) carries AFF2 KO mutation in both alleles. Sequencing results confirmed that this isogenic iPSC line has a stable homozygous 94 bp deletion in the exon 8 of AFF2 gene. The morphology of this human iPS cell line is identical to that of human ES cells. The cells also express the pluripotency markers TRA-1-60, SSEA-3 and Oct4, and demonstrate strong endogenous alkaline phosphatase activity.
|Product Name||AFF2 Knock-out Human Induced Pluripotent Stem Cells (Episomal, HFF)|
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|Storage and Stability||
Store in vapor phase of liquid nitrogen immediately upon receipt. This product is stable for 6 months when stored as directed
Human iPS cells were grown in feeder free conditions with mTeSR1 medium. Each lot of human iPS cells is tested for growth and viability following recovery from cryopreservation. In addition, each lot is tested for expression of TRA-1-60 and OCT4, as well as the activity of alkaline phosphatase.
For Research Use Only. Not for use in diagnostic or therapeutic procedures.