TMEM126B KO cell line available to order. Free of charge wild type control provided. Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3.
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Key facts
- Cell type
- HEK-293T
- Species or organism
- Human
- Tissue
- Kidney
- Form
- Liquid
- Knockout validation
- Sanger Sequencing
- Mutation description
- Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3
Alternative names
HT007, MGC111203, Transmembrane protein 126B
Recommended products
TMEM126B KO cell line available to order. Free of charge wild type control provided. Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3.
Key facts
- Cell type
- HEK-293T
- Species or organism
- Human
- Tissue
- Kidney
- Form
- Liquid
- Knockout validation
- Sanger Sequencing
- Mutation description
- Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3
- Concentration
Properties
- Gene name
- TMEM126B
- Gene editing type
- Knockout
- Gene editing method
- CRISPR technology
- Knockout validation
- Sanger Sequencing
- Zygosity
- Homozygous
Quality control
- STR analysis
- CSF1PO, D13S317, D7S820, D5S818, TH01, D16S539, TPOX
Cell culture
- Biosafety level
- EU: 2 US: 2
- Adherent/suspension
- Adherent
- Gender
- Female
Handling procedures
- Initial handling guidelines
Upon arrival, the vial should be stored in liquid nitrogen vapor phase and not at -80°C. Storage at -80°C may result in loss of viability.
1. Thaw the vial in 37°C water bath for approximately 1-2 minutes.
2. Transfer the cell suspension (0.8 mL) to a 15 mL/50 mL conical sterile polypropylene centrifuge tube containing 8.4 mL pre-warmed culture medium, wash vial with an additional 0.8 mL culture medium (total volume 10 mL) to collect remaining cells, and centrifuge at 201 x g (rcf) for 5 minutes at room temperature. 10 mL represents minimum recommended dilution. 20 mL represents maximum recommended dilution.
3. Resuspend the cell pellet in 5 mL pre-warmed culture medium and count using a haemocytometer or alternative cell counting method seed all remaining cells into a T25.
4. Incubate the culture at 37°C incubator with 5% CO2. Check the culture one day after revival and continue to check until 80% confluent. Media change can be given if needed.
5. Once confluent passage into an appropriate flask at a density of 2x104 cells/cm2. Seeding density is given as a guide only and should be scaled to align with individual lab schedules. Cultures should be monitored daily.- Subculture guidelines
- All seeding densities should be based on cell counts gained by established methods.
- A guide seeding density of 2x104 cells/cm2 is recommended.
- Cells should be passaged when they have achieved 80-90% confluence.
- Culture medium
- DMEM (High Glucose) + 10% FBS
- Cryopreservation medium
- Cell Freezing Medium-DMSO Serum free media, contains 8.7% DMSO in MEM supplemented with methyl cellulose.
Storage
- Shipped at conditions
- Dry Ice
- Appropriate short-term storage conditions
- -196°C
- Appropriate long-term storage conditions
- -196°C
Notes
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 and patent pages.
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
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.
- Associated diseases and disorders
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.
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1 product image
Sanger Sequencing - Human TMEM126B knockout HEK-293T cell line (ab266199)
Homozygous: 1 bp insertion in exon 3
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