产品货号 :  mlR18644

英文名称 :  MAN1B1

中文名称 :  内质网α-甘露糖苷酶1抗体

别    名 :  Alpha 1 2 mannosidase; Endoplasmic reticulum alpha mannosidase 1; Endoplasmic reticulum mannosyl oligosaccharide 1 2 alpha mannosidase 1; Endoplasmic reticulum mannosyl oligosaccharide 1 2 alpha mannosidase; ER alpha 1 2 mannosidase; Man9GlcNAc2 specific processing alpha mannosidase; MANA ER; Mannosidase alpha class 1B member 1.  

研究领域 :  细胞生物  免疫学  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :  Human, Mouse, Rat, Pig, Horse,

产品应用 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  80kDa

细胞定位 :  细胞浆 细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human MAN1B1:211-320/699

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.

PubMed :  PubMed

产品介绍 :   This gene encodes an enzyme belonging to the glycosyl hydrolase 47 family. This enzyme functions in N-glycan biosynthesis, and is a class I alpha-1,2-mannosidase that specifically converts Man9GlcNAc to Man8GlcNAc isomer B. It is required for N-glycan trimming to Man5-6GlcNAc2 in the endoplasmic-reticulum-associated degradation pathway. Mutations in this gene cause autosomal-recessive intellectual disability. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 11. [provided by RefSeq, Dec 2011]

Function:

Involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man9GlcNAc2 to produce Man8GlcNAc2, but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man5-6GlcNAc2.

Subcellular Location:

Endoplasmic reticulum membrane; Single-pass type II membrane protein.

Tissue Specificity:

Widely expressed.

DISEASE:

Mental retardation, autosomal recessive 15 (MRT15) [MIM:614202]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. Note: The disease is caused by mutations affecting the gene represented in this entry.

Similarity:

Belongs to the glycosyl hydrolase 47 family.

SWISS:

Q9UKM7

Gene ID:

11253

Important Note:

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