GCS1 polyclonal antibody
产品名称： GCS1 polyclonal antibody
英文名称： GCS1 polyclonal antibody
产品编号： PAB4280
产品价格： null
产品产地： 台湾
品牌商标： Abnova
更新时间： null
使用范围：
亚诺法生技股份有限公司（Abnova)
- 联系人 :
- 地址 : 台湾台北市内湖区洲子街 108 号 9 楼
- 邮编 : 11493
- 所在区域 : 台湾
- 电话 : +886-920**1152 点击查看
- 传真 : 点击查看
- 邮箱 : sales@abnova.com.tw
- Specification
- Product Description:
- Rabbit polyclonal antibody raised against synthetic peptide of GCS1.
- Immunogen:
- A synthetic peptide (conjugated with KLH) corresponding to C-terminus of human GCS1.
- Host:
- Rabbit
- Reactivity:
- Human, Mouse
- Form:
- Liquid
- Purification:
- Protein G purification
- Storage Buffer:
- In PBS (0.09% sodium azide)
- Storage Instruction:
- Store at 4°C. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.
- Recommend Usage:
- ELISA (1:1000)
Western Blot (1:100-500)
The optimal working dilution should be determined by the end user.
- Note:
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- Publication Reference
- 1.
- Processing of N-linked carbohydrate chains in a patient with glucosidase I deficiency (CDG type IIb).
Volker C, De Praeter CM, Hardt B, Breuer W, Kalz-Fuller B, Van Coster RN, Bause E.Glycobiology. 2002 Aug;12(8):473-83.
- 2.
- A novel disorder caused by defective biosynthesis of N-linked oligosaccharides due to glucosidase I deficiency.
De Praeter CM, Gerwig GJ, Bause E, Nuytinck LK, Vliegenthart JF, Breuer W, Kamerling JP, Espeel MF, Martin JJ, De Paepe AM, Chan NW, Dacremont GA, Van Coster RN.Am J Hum Genet. 2000 Jun;66(6):1744-56. Epub 2000 Apr 28.
- 3.
- Cloning and expression of glucosidase I from human hippocampus.
Kalz-Fuller B, Bieberich E, Bause E.Eur J Biochem. 1995 Jul 15;231(2):344-51.
- Entrez GeneID:
- 7841
- Protein Accession#:
- GCS1_HUMAN
- Gene Name:
- GCS1
- Gene Alias:
- -
- Gene Description:
- glucosidase I
- Gene Ontology:
- Hyperlink
- Gene Summary:
- This gene encodes the first enzyme in the N-linked oligosaccharide processing pathway. The enzyme cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. This protein is located in the lumen of the endoplasmic reticulum. Defects in this gene are a cause of type IIb congenital disorder of glycosylation (CDGIIb). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq
- Other Designations:
- mannosyl-oligosaccharide glucosidase,processing A-glucosidase I
- Gene Pathway