SDHAF1
SDHAF1,即琥珀酸脫氫酶復合物組裝因子1,別名 LYR motif containing 8、LYR motif-containing protein 8、LYRM8、mitochondrial、SDH assembly factor 1。它是一種位于線粒體中的蛋白,由SDHAF1基因編碼,對琥珀酸脫氫酶(SDH)復合物的組裝至關重要,但并不與復合物在體內物理結合。SDHAF1的作用機制涉及促進SDH復合物中鐵硫蛋白亞基SDHB的成熟,保護其不受氧化劑的有害影響。SDHAF1對線粒體呼吸鏈中SDH復合物組裝的調控作用,SDH復合物是線粒體呼吸鏈的關鍵組成部分,參與三羧酸循環和電子傳遞鏈,將琥珀酸氧化為延胡索酸,同時將泛醌還原為泛醌醇。SDHAF1的突變與SDH缺陷型嬰兒白質腦?。ㄒ环N線粒體復合物II缺陷疾?。┫嚓P,該病是一種罕見的遺傳性代謝疾病,主要影響中樞神經系統。因此,SDHAF1是線粒體生物學和相關疾病研究中的一個重要分子靶點。
熱銷產品
SDHAF1 Antibody (CSB-PA406800LA01HU)
驗證數據

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

Immunohistochemistry of paraffin-embedded human placenta tissue using CSB-PA406800LA01HU at dilution of 1:100
SDHAF1 Antibodies
SDHAF1 for Homo sapiens (Human)
產品貨號 | 產品名稱 | 種屬反應性 | 應用類型 |
---|---|---|---|
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)
產品貨號 | 產品名稱 | 來源 |
---|---|---|
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 |