SFXN1は,一炭素代謝に必要なミトコンドリアのセリントランスポーターである
Nora Kory1,2,3,4, Gregory A Wyant1,2,3,4, Gyan Prakash1
1Whitehead Institute for Biomedical Research, 455 Main Street, Cambridge, MA 02142, USA.
まとめ
シデロフレキシン1 (SFXN1) は,核酸とピュリンの合成に不可欠な,一炭素代謝に不可欠なミトコンドリアのセリントランスポーターとして特定されています. 欠乏すると,グリシンとピュリンの生成が妨げられます.
科学分野:
- 細胞生物学
- 代謝経路
- 分子遺伝学
背景:
- 一酸化炭素代謝は 核酸のような重要な代謝物質の合成に不可欠です
- ミトコンドリアへのセリンの侵入は,この経路の重要な,しかし機械的に不明なステップです.
- 内 mitochondrial 膜のタンパク質である sideroflexin 1 (SFXN1) の機能は以前は知られていなかった.
研究 の 目的:
- ミトコンドリアの単炭素代謝経路に関与する遺伝子を特定する.
- 細胞代謝におけるシデロフレキシン1 (SFXN1) の機能を明らかにする.
- ミトコンドリアへのセリン輸送の仕組みを理解する
主な方法:
- CRISPRベースのヒト細胞の遺伝子スクリーニング
- グリシンとピュリンの合成における細胞欠陥の分析.
- 精製されたSFXN1タンパク質を用いた in vitro 輸送測定法
主要な成果:
- SFXN1はミトコンドリアの1炭素代謝経路における重要な遺伝子として特定された.
- SFXN1が欠けている細胞は,グリシンとピュリンの合成に欠陥がある.
- SFXN1はミトコンドリアのセリントランスポーターとしてin vitroで機能することが確認された.
- SFXN1とその同型SFXN3の喪失は重度のグリシンオクストロフィーを引き起こした.
結論:
- SFXN1は,一炭素代謝に不可欠なミトコンドリアのセリントランスポーターです.
- SFXN1は,ニュクレオチドとピュリンの生物合成において重要な役割を果たします.
- この発見はミトコンドリアへのセリン輸送の鍵となるメカニズムを明らかにした.
関連する概念動画
Carbon Dioxide Transport in the Blood
5.1K
Carbon dioxide (CO2) transport in the blood is critical to human physiology. On average, our body cells produce around 200 mL of CO2 per minute, precisely the quantity expelled by the lungs. This process involves the transportation of CO2 from the tissue cells to the lungs in three primary forms.
Forms of CO2 Transport
1. Dissolved in plasma: A small percentage (7-10%) of CO2 is transported and dissolved directly in the plasma.
2. Carbaminohemoglobin: Just over 20% of CO2 is chemically bound to...
Forms of CO2 Transport
1. Dissolved in plasma: A small percentage (7-10%) of CO2 is transported and dissolved directly in the plasma.
2. Carbaminohemoglobin: Just over 20% of CO2 is chemically bound to...
5.1K
Protein Transport into the Inner Mitochondrial Membrane
5.0K
Nuclear encoded mitochondrial precursors are imported to the inner membrane in a multistep process involving two separate translocons, TIM22 and TIM23. TIM23 is a cation-selective pore that remains closed by the N terminal segment of the protein. Negative charges on the TIM23 act as a receptor for the incoming precursor, pulling the positively charged matrix-targeting sequence for peptide insertion and translocation.
Transport of mitochondrial precursors across the TIM23 channel is driven by...
Transport of mitochondrial precursors across the TIM23 channel is driven by...
5.0K
Animal Mitochondrial Genetics
9.2K
Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
9.2K
What is Metabolism?
131.8K
Overview
131.8K
Electron Transport Chains
112.2K
The final stage of cellular respiration is oxidative phosphorylation that consists of two steps: the electron transport chain and chemiosmosis. The electron transport chain is a set of proteins found in the inner mitochondrial membrane in eukaryotic cells. Its primary function is to establish a proton gradient that can be used during chemiosmosis to produce ATP and generate electron carriers, such as NAD+ and FAD, that are used in glycolysis and the citric acid cycle.
The ETC is comprised of...
The ETC is comprised of...
112.2K
Facilitated Transport
148.1K
The chemical and physical properties of plasma membranes cause them to be selectively permeable. Since plasma membranes have both hydrophobic and hydrophilic regions, substances need to be able to transverse both regions. The hydrophobic area of membranes repels substances such as charged ions. Therefore, such substances need special membrane proteins to cross a membrane successfully. In facilitated transport, also known as facilitated diffusion, molecules and ions travel across a...
148.1K


