



Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
LONP1 Double Nickase Plasmid (m) | sc-428748-NIC | 20 µg | $410.00 | |||
LONP1 Double Nickase Plasmid (m2) | sc-428748-NIC-2 | 20 µg | $410.00 |
Lonp1 encodes LONP1, an ATP-dependent serine protease/chaperone localized to the mitochondrial matrix that governs mitochondrial proteostasis by refolding or degrading damaged, misfolded, and oxidized proteins. By controlling turnover of respiratory chain components and regulatory factors, LONP1 supports oxidative phosphorylation, mitochondrial DNA maintenance, and stress-adaptive programs such as the mitochondrial unfolded protein response. Disruption of LONP1 function perturbs mitochondrial dynamics, elevates reactive oxygen species, and compromises bioenergetic homeostasis, linking this pathway to mitochondrial dysfunction phenotypes relevant to neurodegeneration, cardiometabolic stress, and cancer-associated metabolic remodeling. Mouse Lonp1 is therefore a key node for studying quality control mechanisms that integrate proteolysis, ATP-dependent remodeling, and redox signaling in mitochondria.
LONP1 Double Nickase Plasmid (m) consists of a matched pair of plasmids engineered for high-specificity editing of the Lonp1 locus in mouse cell lines. Each plasmid expresses a Cas9 D10A nickase and a distinct sgRNA targeting opposite DNA strands within Lonp1. 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 Lonp1 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 Lonp1-disrupted clones.
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.