Abcam, ab263387, Human TMEM126A knockout HeLa cell lysate

Size: 1Kit
TMEM126A KO cell lysate available now. KO validated by. Free of charge wild type control included. Knockout achieved by using CRISPR/Cas9, 1 bp insertion in exon2 and Insertion of the selection cassette in exon2.
Key facts
Cell type:HeLa,
Species or organism:Human,
Tissue:Cervix,
Knockout validation:Sanger Sequencing,
Mutation description:Knockout achieved by using CRISPR/Cas9, 1 bp insertion in exon2 and Insertion of the selection cassette in exon2.,
Disease:Adenocarcinoma

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, 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-TMEM126A, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, 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.
TMEM126A also known as Transmembrane Protein 126A is a mitochondrial protein with an approximate mass of 26 kDa. This protein is integral to the mitochondrial inner membrane where it plays a role in maintaining mitochondrial function. TMEM126A is expressed in many tissues but shows high levels in energy-demanding tissues like heart and skeletal muscle.
Biological function summary
TMEM126A contributes to the stability and assembly of the mitochondrial respiratory chain Complex I. It forms part of the mitochondrial contact site and cristae organizing system (MICOS) essential for maintaining the inner membrane architecture. By assisting in Complex I assembly TMEM126A supports efficient cellular respiration and energy production which is critical for cell survival and activity.
Pathways
The role of TMEM126A is significant in oxidative phosphorylation and mitochondrial respiration pathways. It interacts closely with Complex I components like NDUFAF1 and NDUFS1. These interactions are vital for proper electron transport chain function impacting cellular energy homeostasis and metabolic regulation.
TMEM126A mutations relate to mitochondrial complex I deficiency and optic atrophy type 7. Complex I deficiency affects energy production leading to neurological and muscular diseases. In optic atrophy type 7 faulty mitochondrial function from TMEM126A links with vision loss. TMEM126A works alongside proteins such as OPA1 and OXPHOS components in these conditions highlighting its importance in mitochondrial health.