MAN1B1 Antibody

說明書

中文名稱：

MAN1B1兔多克隆抗體
貨號：

CSB-PA006800
規(guī)格：

￥1090
圖片：
Western Blot analysis of 3T3 cells using MAN1B1 Polyclonal Antibody
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

Uniprot No.：

Q9UKM7
基因名：

MAN1B1
別名：

MAN1B1 antibody; UNQ747/PRO1477Endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase antibody; EC 3.2.1.113 antibody; ER alpha-1,2-mannosidase antibody; ER mannosidase 1 antibody; ERMan1 antibody; Man9GlcNAc2-specific-processing alpha-mannosidase antibody; Mannosidase alpha class 1B member 1 antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Synthesized peptide derived from the Internal region of Human MAN1B1.
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

Non-conjugated
抗體亞型：

IgG
純化方式：

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

WB, ELISA

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
ELISA	1:10000

Protocols：

Western Blotting (WB) Protocol
ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

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).
基因功能參考文獻(xiàn)：
1. 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
2. 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
3. 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
4. MAN1B1 defective congenital disorder of glycosylation is reviewed. PMID: 26577042
5. 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
6. Results suggest that endoplasmic reticulum mannosidase I (ERManI) is turned over by an active autophagic process. PMID: 25411339
7. 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
8. we linked mutations in MAN1B1 to a Golgi glycosylation disorder. Additionally, our results support the recent findings on MAN1B1 localization PMID: 24348268
9. MAN1B1 deficiency appeared to be a frequent cause in our cohort of patients with unsolved congenital disorder of glycosylation type II. PMID: 24566669
10. 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
11. 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
12. Golgi localization of ERManI defines spatial separation of the mammalian glycoprotein quality control system PMID: 21697506
13. A homozygous nonsense mutation in MAN1B1 segregated with nonsyndromic autosomal-recessive intellectual disability and additional dysmorphic features. PMID: 21763484
14. ER processing alpha1,2-mannosidase (ER ManI) has a role in ER-associated degradation of misfolded proteins PMID: 12736254
15. Modification by endoplasmic reticulum mannosidase I (ERManI) contributes to the preferential selection of the misfolded AAT monomer for proteasomal degradation. PMID: 12815101
16. glycoside bond cleavage proceeds through a least motion conformational twist of a properly predisposed substrate PMID: 15713668
17. Increased activity of alpha-mannosidase in the peritoneal fluid is associated with gynecologic cancers and pelvic inflammatory disease PMID: 15785934
18. 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
19. ERManI is required for trimming to Man(5-6)GlcNAc(2) and for endoplasmic reticulum associated degradation in cells in vivo. PMID: 18003979
20. the true catalytic proton donor is Asp463 in the human endoplasmic reticulum alpha-(1-->2)-mannosidase I PMID: 18619586
21. 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(guān)疾病：

Mental retardation, autosomal recessive 15 (MRT15)
亞細(xì)胞定位：

Endoplasmic reticulum membrane; Single-pass type II membrane protein.
蛋白家族：

Glycosyl hydrolase 47 family
組織特異性：

Widely expressed.
數(shù)據(jù)庫鏈接：

HGNC: 6823

OMIM: 604346

KEGG: hsa:11253

STRING: 9606.ENSP00000360645

UniGene: Hs.279881

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