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AB259185

人TMEM126B knockout HEK-293T cell裂解物

Human TMEM126B knockout HEK-293T cell lysate

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TMEM126B KO cell lysate available now. KO validated. Free of charge wild type control included. Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3.

查看别名

HT007, MGC111203, Transmembrane protein 126B

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Sanger Sequencing - Human TMEM126B knockout HEK-293T cell lysate (AB259185)
  • Sanger seq

Unknown

Sanger Sequencing - Human TMEM126B knockout HEK-293T cell lysate (AB259185)

Homozygous : 1 bp insertion in exon 3

关键信息

细胞类型

HEK-293T

种属

Human

组织

Kidney

敲除验证

Sanger Sequencing

突变描述

Knockout achieved by using CRISPR/Cas9, Homozygous: 1 bp insertion in exon 3.

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产品详情

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 and patent pages.

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规格

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性能和储存信息

基因名称
TMEM126B
基因编辑类型
Knockout
基因编辑方法
CRISPR technology
敲除验证
Sanger Sequencing
合子性
Homozygous
运输条件
Ambient - Can Ship with Ice
推荐的短期储存条件
-20°C
推荐的长期储存条件
-20°C
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补充信息

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.
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质量控制

STR 分析

CSF1PO, D13S317, D7S820, D5S818, TH01, D16S539, TPOX

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细胞培养

生物安全等级

EU: 2 US: 2

贴壁/悬浮

Adherent

性别

Female

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产品实验方案

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