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Rabbit Anti-MAN1B1/AF555 Conjugated antibody (bs-18644R-AF555)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-18644R-AF555
英文名称 Anti-MAN1B1/AF555
中文名称 AF555标记的内质网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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
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 (predicted: Human, Mouse, Rat, Pig, Horse, )
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 80kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MAN1B1
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:
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.

Database links:

Entrez Gene: 11253 Human

Entrez Gene: 227619 Mouse

Omim: 604346 Human

SwissProt: Q9UKM7 Human

Unigene: 591887 Human



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