内质网α-甘露糖苷酶1抗体
规格：1mg/1ml
英文名： MAN1B1
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 manno
分子量： 80kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MAN1B1
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆 细胞膜
内质网α-甘露糖苷酶1抗体产品介绍：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 内质网α-甘露糖苷酶1抗体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 内质网α-甘露糖苷酶1抗体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.
内质网α-甘露糖苷酶1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid