Anti-PMM2/FITC Conjugated抗体-抗体-抗体-生物在线
Anti-PMM2/FITC Conjugated抗体

Anti-PMM2/FITC Conjugated抗体

商家询价

产品名称: Anti-PMM2/FITC Conjugated抗体

英文名称: Anti-PMM2/FITC

产品编号: YB--12741R-FITC

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

上海钰博生物科技有限公司
  • 联系人 : 陈环环
  • 地址 : 上海市沪闵路6088号龙之梦大厦8楼806室
  • 邮编 : 200612
  • 所在区域 : 上海
  • 电话 : 183****2235
  • 传真 : 021-60514606
  • 邮箱 : shybio@126.com

 Anti-PMM2/FITC Conjugated抗体

产品编号 YB-12741R-FITC
英文名称 Anti-PMM2/FITC
中文名称 FITC标记的磷酸甘露糖变位酶2抗体
别    名 AI585868; BOS_22465; C86848; CDG 1; CDG1; CDG1a; CDGS; MGC127449; Phosphomannomutase 2; PMM 2; Pmm2; PMM2_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Cow, Sheep, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 28kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PMM2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
The protein encoded by this gene catalyzes the isomerization of mannose 6-phosphate to mannose 1-phosphate, which is a precursor to GDP-mannose necessary for the synthesis of dolichol-P-oligosaccharides. Mutations in this gene have been shown to cause defects in glycoprotein biosynthesis, which manifests as carbohydrate-deficient glycoprotein syndrome type I. [provided by RefSeq, Jul 2008]

Function:
Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions.

Subcellular Location:
Cytoplasm.

DISEASE:
Defects in PMM2 are the cause of congenital disorder of glycosylation type 1A (CDG1A) [MIM:212065]; also known as carbohydrate-deficient glycoprotein syndrome type Ia (CDGS1A) or Jaeken syndrome. Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1A is an autosomal recessive disorder characterized by a severe encephalopathy with axial hypotonia, abnormal eye movement, and pronounced psychomotor retardation, as well as peripheral neuropathy, cerebellar hypoplasia, and retinitis pigmentosa. Patients show a peculiar distribution of subcutaneous fat, nipple retraction, and hypogonadism.

Similarity:
Belongs to the eukaryotic PMM family.

Database links:

Entrez Gene: 510978 Cow

Entrez Gene: 5373 Human

Entrez Gene: 54128 Mouse

Entrez Gene: 302915 Rat

Omim: 601785 Human

SwissProt: Q3SZJ9 Cow

SwissProt: O15305 Human

SwissProt: Q9Z2M7 Mouse

Unigene: 625732 Human

Unigene: 9699 Mouse

Unigene: 58278 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications