Invitrogen

SURF1 Polyclonal Antibody

Name: SURF1 Polyclonal Antibody
Brand: Invitrogen
SKU: PA5-49828

查看其他7个SURF1抗体

说明书

实验方案

问题与答案

技术支持

说明书

实验方案

问题与答案

技术支持

说明书

实验方案

问题与答案

技术支持

抗体检测数据 (1)

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SURF1 Antibody (PA5-49828) in WB

Western Blot analysis of SURF1 was performed using extracts from Jurkat cells. The membrane was probed with SURF1 Polyclonal Antibody (Product # PA5-49828).

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Immunogen image

Immunogen Image SURF1 Polyclonal Antibody

产品信息

PA5-49828

应用

建议稀释比

已发表文章

免疫印迹 (WB)

1:500-1:3,000

产品规格

种属反应

Human, Mouse, Rat

宿主/亚型

Rabbit / IgG

分类

Polyclonal

类型

Antibody

抗原

Synthesized peptide derived from internal of human SURF1.

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偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

1 mg/mL

纯化类型

Antigen affinity chromatography

保存液

PBS, pH 7.4, with 50% glycerol

内含物

0.02% sodium azide

保存条件

-20°C

运输条件

Ambient (domestic); Wet ice (international)

AB_2635281

产品详细信息

The antibody detects endogenous levels of total SURF-1 protein.

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.

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