Abcam, ab266199, Human TMEM126B knockout HEK-293T cell line

Size: 2 x 1000000Cells / vial / 1000000Cells / vial
TMEM126B KO cell line available to order. KO validated by. Free of charge wild type control available. Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3. To order both knockout and wild-type control cells: select 2 x 1000000Cells/vial. To order only knockout cells: select 1000000Cells/vial.
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 3

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-TMEM126B, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Zygosity-Homozygous, 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.
TMEM126B also known as transmembrane protein 126B plays a significant role in the assembly of mitochondrial complex I. Its molecular weight is approximately 25 kDa. This protein integrates into the inner mitochondrial membrane and expresses in many tissues where it serves as an important element for mitochondrial function. TMEM126B operates as one part of a larger assembly machinery that ensures proper formation of complex I. This process is essential for cellular respiration linking structural components to functionality within the mitochondria.
Biological function summary
This protein participates in the assembly of the mitochondrial membrane protein complex. It operates as a member of the mitochondrial complex I assembly (MCIA) complex which comprises several other assembly factors. These factors collaborate to promote efficient positioning and stabilization of complex I within the mitochondrial membrane. This process ensures adequate electron transport chain function which is vital for ATP production and overall energy metabolism.
Pathways
TMEM126B is essential for mitochondrial respiratory function specifically in the oxidative phosphorylation pathway. This pathway involves multiple steps for the efficient production of ATP. TMEM126B works alongside proteins such as NDUFAF1 and ECSIT facilitating complex assembly to smooth the transfer of electrons from NADH to ubiquinone. The protein contributes to the function of the electron transport chain and uncouples energy production from heat release impacting cellular energy efficiency.
Malfunctioning of TMEM126B has strong associations with mitochondrial complex I deficiency a condition affecting energy metabolism in cells. Dysfunction in complex I can lead to neurodegenerative diseases such as Leigh syndrome. Additionally altered TMEM126B expression may influence disease progression by affecting proteins linked to energy metabolism such as ATP synthase. Understanding these relationships opens avenues for potential therapeutic strategies targeting mitochondrial dysfunction.