ChiPS31

Description

Organism: Homo sapiens, human

Cell Type: induced pluripotent stem cell

Source:  fibroblast line HD509 of an adult rare homozygous HD individual

Gender: male

Age: 59 year-old

Disease: Huntington’s Disease

Haplotype: 42/44 CAG repeats on each allele

Delivery system: infection with an improved polycistronic lentivirus

Reprogramming factors: OCT4, SOX2 and KLF4 (OSK)

Datasheet: available under request

References: 

1. Camnasio S, Delli Carri A, Lombardo A, Grad I, Mariotti C, Castucci A, Rozell B, Lo Riso P, Castiglioni V, Zuccato C, Rochon C, Takashima Y, Diaferia G,Biunno I, Gellera C, Jaconi M, Smith A, Hovatta O, Naldini L, Di Donato S, Feki A, Cattaneo E. The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington’s disease patients demonstrates mutation related enhanced lysosomal activity. Neurobiol Dis. 2012 Apr;46(1):41-51.

2. Baronchelli S, La Spada A, Ntai A, Barbieri A, Conforti P, Jotti GS, Redaelli S, Bentivegna A, De Blasio P, Biunno I. Epigenetic and transcriptional modulation of WDR5, a chromatin remodeling protein, in Huntington’s disease human induced pluripotent stem cell (hiPSC) model. Mol Cell Neurosci. 2017 May 2;82:46-57.