Description
Key facts
Cell type:HEK-293T,
Species or organism:Human,
Tissue:Kidney,
Form:LiquidSee storage information,
Knockout validation:Sanger Sequencing,
Mutation description:Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 4
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-ECI2, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
PECI also known as ECI2 or peroxisomal delta(3)delta(2)-enoyl-CoA isomerase plays an important role in the beta-oxidation of unsaturated fatty acids. This enzyme with a molecular weight of approximately 33 kDa catalyzes the conversion of cis-double bonds in unsaturated fatty acyl-CoAs turning them into trans-double bonds which are useful for subsequent beta-oxidation. PECI primarily locates to the peroxisomes and is expressed in tissues with high metabolic rates like liver and heart.
Biological function summary
PECI contributes to the metabolism of fatty acids by ensuring effective breakdown of unsaturated fatty acids in peroxisomes. The enzyme participates as a solitary player and not part of a larger protein complex. By converting cis-double bonds to the trans configuration PECI aids in stabilizing the substrate for further catabolic processing. This facilitated breakdown is critical for energy production especially during fasting when fatty acids become a significant energy source.
Pathways
PECI's mechanical function integrates into the larger pathway of fatty acid metabolism especially the peroxisomal beta-oxidation pathway. This pathway teams with enzymes such as acyl-CoA oxidase and multifunctional enzyme protein to facilitate complete fatty acid degradation. PECI's transformation of unsaturated fatty acids ensures these substrates enter the pathway's main cycle aiding in continued lipid metabolism and maintaining cellular energy balance.
Disruptions in PECI function might lead to metabolic conditions like X-linked adrenoleukodystrophy or Refsum disease which both involve impaired fatty acid oxidation. The enzyme interacts with proteins like ABCD1 a transporter associated with peroxisomal biogenesis which holds significance in adrenoleukodystrophy. Proper function and regulation of PECI remain important for managing these disorders and ensuring uninterrupted fatty acid disassembly for cellular energy requirements.
