MAN1B1 Antibody
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中文名稱：MAN1B1兔多克隆抗體
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貨號：CSB-PA217897
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規格：￥2024
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圖片：
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Western blot analysis of extracts from Hala cells, using MAN1B1 antibody.
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) MAN1B1 Polyclonal antibody
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Uniprot No.：
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基因名：
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宿主：Rabbit
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反應種屬：Human
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免疫原：Synthesized peptide derived from internal of Human MAN1B1.
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免疫原種屬：Homo sapiens (Human)
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克隆類型：Polyclonal
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純化方式：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度：It differs from different batches. Please contact us to confirm it.
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產品提供形式：Liquid
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應用范圍：ELISA,WB
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推薦稀釋比：
Application Recommended Dilution WB 1:500-1:3000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：Involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man(9)GlcNAc(2) to produce Man(8)GlcNAc(2), but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man(5-6)GlcNAc(2).
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基因功能參考文獻：
- Results show that MAN1B1 expression was higher in bladder cancer (BC) tissues than those in normal tissues. Its overexpression was associated with poor prognosis. Furthermore, MAN1B1 seems to act as an oncogene in BC, which improved the likelihood of MAN1B1 taking on a promising prognostic biomarker. PMID: 30218751
- The two genetic associations with severe liver disease that had been suspected previously (one SNP for SERPINA1 and another for MAN1B1) were not confirmed in our cohort. For MAN1B1, four major haplotypes were identified but the prevalence of PHT did not significantly differ between them. PMID: 28887821
- in the bound substrate complex, hydrophobic stacking interactions between Trp residues and the glycan core anchor the base of the glycan structure to the enzyme cleft PMID: 27856750
- MAN1B1 defective congenital disorder of glycosylation is reviewed. PMID: 26577042
- Data show that HIV-1 env protein interacts with alpha-mannosidases ERManI (MAN1B1) and initiates endoplasmic reticulum (ER)-associated protein degradation (ERAD) pathway degradation process. PMID: 26205822
- Results suggest that endoplasmic reticulum mannosidase I (ERManI) is turned over by an active autophagic process. PMID: 25411339
- The properties of ERMAN1 enable rapid selection of endoplasmic reticulum-associated degradation substrates in the endoplasmic reticulum.ERMAN1 digests mono-, di- and tri-glucosylated N-glycans. PMID: 24519966
- we linked mutations in MAN1B1 to a Golgi glycosylation disorder. Additionally, our results support the recent findings on MAN1B1 localization PMID: 24348268
- MAN1B1 deficiency appeared to be a frequent cause in our cohort of patients with unsolved congenital disorder of glycosylation type II. PMID: 24566669
- A novel post-transcriptional regulatory mechanism for ERManI via miR-125b and this molecule contributes to the regulation of carcinogenesis in hepatocellular carcinoma. PMID: 23940818
- ERManI and gamma-COP contribute to a golgi-based quality control module that facilitates the retrieval of captured ERAD substrates back to the endoplasmic reticulum. PMID: 23427261
- Golgi localization of ERManI defines spatial separation of the mammalian glycoprotein quality control system PMID: 21697506
- A homozygous nonsense mutation in MAN1B1 segregated with nonsyndromic autosomal-recessive intellectual disability and additional dysmorphic features. PMID: 21763484
- ER processing alpha1,2-mannosidase (ER ManI) has a role in ER-associated degradation of misfolded proteins PMID: 12736254
- Modification by endoplasmic reticulum mannosidase I (ERManI) contributes to the preferential selection of the misfolded AAT monomer for proteasomal degradation. PMID: 12815101
- glycoside bond cleavage proceeds through a least motion conformational twist of a properly predisposed substrate PMID: 15713668
- Increased activity of alpha-mannosidase in the peritoneal fluid is associated with gynecologic cancers and pelvic inflammatory disease PMID: 15785934
- overexpression of Golgi alpha1,2-mannosidase IA, IB, and IC also accelerates ERAD of terminally misfolded human alpha1-antitrypsin variant null (Hong Kong) (NHK), and mannose trimming from the N-glycans on NHK in 293 cells PMID: 17727818
- ERManI is required for trimming to Man(5-6)GlcNAc(2) and for endoplasmic reticulum associated degradation in cells in vivo. PMID: 18003979
- the true catalytic proton donor is Asp463 in the human endoplasmic reticulum alpha-(1-->2)-mannosidase I PMID: 18619586
- the identified single-nucleotide polymorphism can accelerate the onset of the end-stage liver disease associated with alpha1-antitrypsin deficiency and underscore the contribution of biosynthetic quality control as a modifier of genetic disease PMID: 19444872
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相關疾病：Mental retardation, autosomal recessive 15 (MRT15)
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亞細胞定位：Endoplasmic reticulum membrane; Single-pass type II membrane protein.
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蛋白家族：Glycosyl hydrolase 47 family
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組織特異性：Widely expressed.
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數據庫鏈接：
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