



Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
PMPCB Double Nickase Plasmid (h) | sc-405207-NIC | 20 µg | $410.00 |
PMPCB encodes the catalytic beta subunit of the mitochondrial processing peptidase (MPP), a key protease that removes N-terminal targeting presequences from nuclear-encoded proteins after their import into the mitochondrial matrix. This processing step is essential for mitochondrial protein maturation, respiratory chain biogenesis, and maintenance of oxidative phosphorylation and metabolic homeostasis. PMPCB function intersects with mitochondrial import and proteostasis pathways, including coordination with TIM/TOM translocases and matrix chaperones that guide precursor folding and assembly. Disruption of mitochondrial presequence processing is implicated in mitochondrial dysfunction phenotypes and has been associated with neurodevelopmental and neuromuscular disease mechanisms linked to impaired energy metabolism.
PMPCB Double Nickase Plasmid (h) consists of a matched pair of plasmids engineered for high-specificity editing of the PMPCB locus in human cell lines. Each plasmid expresses a Cas9 D10A nickase and a distinct sgRNA targeting opposite DNA strands within PMPCB. 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 PMPCB 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 PMPCB-disrupted clones.
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.