Invitrogen

GCS1 Polyclonal Antibody

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GCS1 Antibody (PA5-28312) in WB

Western Blot using GCS1 Polyclonal Antibody (Product # PA5-28312). Sample (30 µg whole cell lysate). A: H1299. 7.5% SDS PAGE. GCS1 Polyclonal Antibody (Product # PA5-28312) diluted at 1:1,000.

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产品信息

PA5-28312

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

宿主/亚型

Rabbit / IgG

分类

Polyclonal

类型

Antibody

抗原

Recombinant fragment corresponding to a region within amino acids 136 and 355 of Human Glucosidase I

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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

0.1M tris glycine, pH 7, with 10% 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

Wet ice

RRID

AB_2545788

产品详细信息

Recommended positive controls: 293T, A431, H1299, HeLaS3, HepG2, Molt-4, Raji.

Predicted reactivity: Mouse (87%).

Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

靶标信息

GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.

⚠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。