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SDHAF1

SDHAF1，即琥珀酸脫氫酶復(fù)合物組裝因子1，別名 LYR motif containing 8、LYR motif-containing protein 8、LYRM8、mitochondrial、SDH assembly factor 1。它是一種位于線(xiàn)粒體中的蛋白，由SDHAF1基因編碼，對(duì)琥珀酸脫氫酶（SDH）復(fù)合物的組裝至關(guān)重要，但并不與復(fù)合物在體內(nèi)物理結(jié)合。SDHAF1的作用機(jī)制涉及促進(jìn)SDH復(fù)合物中鐵硫蛋白亞基SDHB的成熟，保護(hù)其不受氧化劑的有害影響。SDHAF1對(duì)線(xiàn)粒體呼吸鏈中SDH復(fù)合物組裝的調(diào)控作用，SDH復(fù)合物是線(xiàn)粒體呼吸鏈的關(guān)鍵組成部分，參與三羧酸循環(huán)和電子傳遞鏈，將琥珀酸氧化為延胡索酸，同時(shí)將泛醌還原為泛醌醇。SDHAF1的突變與SDH缺陷型嬰兒白質(zhì)腦?。ㄒ环N線(xiàn)粒體復(fù)合物II缺陷疾?。┫嚓P(guān)，該病是一種罕見(jiàn)的遺傳性代謝疾病，主要影響中樞神經(jīng)系統(tǒng)。因此，SDHAF1是線(xiàn)粒體生物學(xué)和相關(guān)疾病研究中的一個(gè)重要分子靶點(diǎn)。

熱銷(xiāo)產(chǎn)品

SDHAF1 Antibody (CSB-PA406800LA01HU)

驗(yàn)證數(shù)據(jù)

CSB-PA406800LA01HU

Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA406800LA01HU at dilution of 1:100

CSB-PA406800LA01HU

Immunohistochemistry of paraffin-embedded human placenta tissue using CSB-PA406800LA01HU at dilution of 1:100

SDHAF1 Antibodies

SDHAF1 for Homo sapiens (Human)

產(chǎn)品貨號(hào) 產(chǎn)品名稱(chēng) 種屬反應(yīng)性 應(yīng)用類(lèi)型
CSB-PA406800LA01HU SDHAF1 Antibody Human ELISA, IHC
CSB-PA406800LB01HU SDHAF1 Antibody, HRP conjugated Human ELISA
CSB-PA406800LC01HU SDHAF1 Antibody, FITC conjugated Human
CSB-PA406800LD01HU SDHAF1 Antibody, Biotin conjugated Human ELISA

SDHAF1 Proteins

SDHAF1 Proteins for Homo sapiens (Human)

產(chǎn)品貨號(hào) 產(chǎn)品名稱(chēng) 來(lái)源
CSB-YP406800HU
CSB-EP406800HU
CSB-BP406800HU
CSB-MP406800HU
CSB-EP406800HU-B
 Recombinant Human Succinate dehydrogenase assembly factor 1, mitochondrial (SDHAF1) Yeast
E.coli
Baculovirus
Mammalian cell
In Vivo Biotinylation in E.coli