헌팅턴 신경세포 | Huntington HTT50CAG cell | 고마바이오텍

특징 

• ioGlutamatergic Neurons HTT 50CAG/WT는 헌팅턴 유전자 50 CAG를 발현하도록 리프로그래밍 된 Glutamatergic neuron 입니다. 
• pan-neuronal protein(MAP2, TUBB2)과 glutamatergic neuron-specific transporter(VGLUT2)를 발현합니다.
  
• 빠르게 성숙하고 structural neuronal network를 11일 안에 형성합니다.
• 대조군인 isogenic control인 wild-type ioGlutamatergic Neurons™에 비해 신경망 형성이 지연되고 자발적인 활동이 감소하는 등, 헌팅턴병 관련 특징을 나타냅니다.
  

적용

• Huntington’s disease research
  
• Drug discovery
• Disease modelling
• Electrophysiological assays (MEA)
• Co-culture studies

참고 데이터

1. ioGlutamatergic Neurons HTT 50CAG/WT generated by transcription factor-driven reprogramming of iPSCs using opti-ox technology 

ioGlutamatergic Neurons HTT 50CAG/WT의 11일간 배양 후 급격한 형태학적 변화를 포착하는 비디오입니다. 

2. ioGlutamatergic Neurons HTT 50CAG/WT express neuron-specific markers with protein expression highly reminiscent to the isogenic control

 

3. ioGlutamatergic Neurons HTT 50CAG/WT form structural networks by day 11

ioGlutamatergic 뉴런 HTT 50CAG/WT는 동질성 대조군과 비교했을 때 빠르게 성숙하고 11일에 걸쳐 구조적 뉴런 네트워크를 형성합니다. (Day 1 to 11 post thawing; 100X magnification.) 

4. Genotype validation of heterozygous 50 CAG repeat expansion

(A) Successful on-target integration into one HTT allele confirmed by gel electrophoresis. Genotyping primers flanking the endogenous HTT CAG repeat expansion region produce a band at approximately 320 bps, by PCR, in both isogenic control (ioGlutamatergic Neurons) and disease model (ioGlutamatergic Neurons HTT 50CAG/WT). PCR fragments at 395 bps detect on-target gene editing and introduction of a 50 CAG repeat expansion in ioGlutamatergic Neurons HTT 50CAG/WT only. (B) Amplicon PCR of the plasmid donor reveals no random integration in genomic DNA from targeted colonies via gel electrophoresis. Off-target random insertion of the donor template (used to introduce the 50 CAG repeat expansion at the WT HTT locus) is detected by PCR amplification of the donor vector backbone. This is not detected in the samples from ioGlutamatergic Neurons HTT 50CAG/WT.