The Progerin/Lamin A qPCR Detection Kit allows the comparative and quantitative detection of progerin expression by Real-Time PCR in cDNA samples and by Real-Time PCR, totally discriminating this specific target from the wild-type form, lamin A. The content of the kit is sufficient for up to 40 determinations, including two calibration curves for lamin A and progerin (quantitative method) and two further amplification mixes for two reference genes necessary for the normalization of the expression data (comparative method).
Progerin/Lamin A qPCR Detection Kit
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare and fatal genetic disease characterized by the onset of premature aging in childhood. The majority of cases is sporadic and is caused by a recurrent dominant de novo point mutation in lmna gene coding for lamin A protein. This mutation constitutively activates a cryptic site that causes the expression of a mutated form of lamin A known as progerin. The possibility to accurately quantify the mutated transcript could give to the researchers the ability to assess the effects of experimental substances in vitro administered to HGPS cells on progerin expression; moreover, researchers could also compare the amount of transcript produced in different cell lines or different growth stages and correlate the production of the mutant protein to the wild type and/or to the progression of the disease. The Progerin/Lamin A qPCR Detection Kit allows two possible methods of analysis: quantitative and comparative.
Two positive standard controls for lamin A and progerin are included in the kit to perform the calibration curves needed to derive the copies number of the two target transcripts in the sample.
The primers included in the optimized Lamin A Master Mix recognize a specific cDNA region that is present in the human lamin A coding sequence and absent in human progerin coding sequence. From this amplification reaction originates a PCR product of 179bp. Optimized Progerin Master Mix includes primers that recognize a specific human cDNA region flanking the deletion of 150 nucleotides that characterized the progerin transcript. From this amplification reaction originates a PCR product of 160bp. Primers design have been accurately designed with the aim of maximizing the ability to discriminate progerin against wild-type lamin A.
|Product type||Diagnostic kit|
|Testing time||2 hours|