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Proteintech
SURF1 Recombinant Rabbit Monoclonal Antibody (230210D5)
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产品信息
83052-7-RR
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse
宿主/亚型
Rabbit
/ IgG
Expression System
HEK293F
分类
Recombinant Monoclonal
类型
Antibody
克隆号
230210D5
抗原
SURF1 fusion protein Ag3959
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Protein A
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Wet ice
产品详细信息
Immunogen sequence: MAAVAALQLG LRAAGLGRAP ASAAWRSVLR VSPRPGVAWR PSRCGSSAAE ASATKAEDDS FLQWVLLLIP VTAFGLGTWQ VQRRKWKLNL IAELESRVLA EPVPLPADPM ELKNLEYRPV KVRGCFDHSK ELYMMPRTMV DPVREAREGG LISSSTQSGA YVVTPFHCTD LGVTILVNRG FVPRKKVNPE TRQKGQIEGE VDLIGMVRLT ETRQPFVPEN NPERNHWHYR DLEAMARITG AEPIFIDANF QSTVPGGPIG GQTRVTLRNE HLQYIVTWYG LSAATSYLWF KKFLRGTPGV
靶标信息
The SURF-1 protein demonstrates a vital role in the assembly of complex IV (CIV or COX) of the mitochondrial respiratory chain. Expressed in the inner mitochondrial membrane, mutations of the SURF-1 gene generally cause cytochrome c oxidase complex IV deficiency. Shortage of complex IV leads to Leigh syndrome, a severe neurological disorder. Leigh syndrome patients are usually subject to rapidly progressive encephalopathy, characterized by necrotic lesions in subcortical brain regions. SURF-1 mutations correlate to high post-implantation embryonic lethality as well as early-onset mortality of post-natal individuals. Considerable deficit in muscle strength and motor performance is also a profound and isolated defect of SURF-1 activity in skeletal muscle and liver. Heart, brain and skeletal muscle morphological abnormalities frequently occur due to SURF-1 mutations.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: Surfeit locus protein 1
基因别名: 0610010F23Rik; CMT4K; SURF-1; SURF1
UniProt ID: (Human) Q15526, (Mouse) P09925
Entrez Gene ID: (Human) 6834, (Mouse) 20930
