



Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
IVD Double Nickase Plasmid (h) | sc-405499-NIC | 20 µg | $410.00 |
Human IVD encodes isovaleryl-CoA dehydrogenase, a mitochondrial flavoprotein that catalyzes a key dehydrogenation step in leucine catabolism within the acyl-CoA dehydrogenase family. By transferring electrons to the electron-transfer flavoprotein system, IVD supports mitochondrial energy metabolism and influences redox homeostasis during amino acid turnover. Loss-of-function variants in IVD are associated with isovaleric acidemia, a disorder characterized by accumulation of isovaleryl-CoA–derived metabolites and secondary mitochondrial stress. In research settings, IVD is used to study branched-chain amino acid metabolism, mitochondrial quality control, and metabolic vulnerability in models of inborn errors of metabolism.
IVD Double Nickase Plasmid (h) consists of a matched pair of plasmids engineered for high-specificity editing of the IVD locus in human cell lines. Each plasmid expresses a Cas9 D10A nickase and a distinct sgRNA targeting opposite DNA strands within IVD. When directed to adjacent sites on opposite DNA strands, the two nickases generate offset single-strand nicks that together produce a staggered double-strand break, requiring coordinated on-target activity from both guides. The resulting DNA break is resolved by endogenous cellular repair pathways, most commonly through non-homologous end joining (NHEJ), leading to insertions or deletions that disrupt IVD function. By requiring dual sgRNA engagement at the target locus, the double nicking approach enhances editing specificity and provides a complementary CRISPR strategy for applications where additional control over targeting precision is desired.
To support efficient identification of edited cells, one plasmid encodes GFP for fluorescent visualization of transfected populations, while the companion plasmid carries a puromycin resistance gene for antibiotic selection. Together, these features support efficient enrichment of co-transfected populations and simplify the validation of IVD-disrupted clones.
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.