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Invitrogen
SURF1 Polyclonal Antibody
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产品信息
PA5-30187
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant protein encompassing a sequence within the center region of human SURF1. The exact sequence is proprietary.
偶联物
形式
Liquid
浓度
0.83 mg/mL
纯化类型
Antigen affinity chromatography
保存液
0.1M tris glycine, pH 7, with 20% glycerol
内含物
0.01% thimerosal
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
Recommended positive controls: Jurkat.
Predicted reactivity: Mouse (84%), Rat (82%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
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: CMT4K; SURF-1; SURF1
UniProt ID: (Human) Q15526
Entrez Gene ID: (Human) 6834
