Description
Key facts
Cell type:HEK-293T,
Species or organism:Human,
Tissue:Kidney,
Knockout validation:Sanger Sequencing,
Mutation description:Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp deletion in exon 1.
Product details:
Knockout cell lysate achieved by CRISPR/Cas9.
REACH authorisation
Abcam has not and does not intend to apply for the REACH Authorisation of customers' uses of products that contain European Authorisation list (Annex XIV) substances.
It is the responsibility of our customers to check the necessity of application of REACH Authorisation, and any other relevant authorisations, for their intended uses.
Lysate preparation:
Our lysates are made using RIPA buffer to which we add a protease inhibitor cocktail and phosphatase inhibitor cocktail (ratio: 300:100:10).
This means that the protein of interest is denatured.
If you require a native form of the protein please use the live cell version. Please refer to our lysis protocol for further details on how our lysates are prepared.
User storage instructions:
Lyophilizate may be stored at 4°C. After reconstitution, store at -20°C for short-term storage or -80°C for long-term storage.
This product is subject to limited use licenses from The Broad Institute and ERS Genomics Limited, and is developed with patented technology. For full details of the limited use licenses and relevant patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-CHCHD3, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Zygosity-Homozygous, Shipped at conditions-Ambient - Can Ship with Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
MIC19 also known as mitofilin is an integral component of the inner mitochondrial membrane part of the mitochondrial inner membrane organizing system (MINOS). It has a molecular mass of approximately 86 kDa. Expression of MIC19 occurs mainly in tissues with high energy demands including the heart and skeletal muscles. It plays a mechanical role in maintaining the structural integrity of mitochondrial cristae by anchoring specific protein components within the membrane.
Biological function summary
MIC19 contributes to the organization and functional maintenance of mitochondrial architecture by participating in the MINOS complex. This complex regulates the interaction between the inner and outer mitochondrial membranes ensuring efficient mitochondrial dynamics and biogenesis. Furthermore MIC19 supports mitochondrial bioenergetics by stabilizing cristae structures necessary for optimal electron transport chain function.
Pathways
MIC19 integrates into the processes controlling mitochondrial fusion and fission dynamics. It functions within the pathways that are essential for cellular energy metabolism and apoptosis. MIC19 influences these pathways through interactions with proteins such as OPA1 which regulates inner membrane fusion and with components of the electron transport chain. This integrative role highlights MIC19's involvement in maintaining cellular energy homeostasis and programmed cell death.
MIC19 associates with neurodegenerative diseases and cardiomyopathies. Altered expression or mutations in MIC19 can disrupt cristae architecture impairing mitochondrial function and energy production. This disruption relates to the disease processes seen in mitochondrial myopathy where proteins like OPA1 also play a role in mediating mitochondrial dysfunction. The interconnected roles of MIC19 and these associated proteins suggest potential therapeutic targets for intervention in mitochondrial-related conditions.
