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MAN1B1 encodes an enzyme belonging to the glycosyl hydrolase 47 family. Zusätzlich bieten wir Ihnen MAN1B1 Proteine (9) und viele weitere Produktgruppen zu diesem Protein an.
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MAN1B1 defective congenital disorder of glycosylation is reviewed.
Data show that HIV-1 env (zeige ERVW-1 Antikörper) protein interacts with alpha-mannosidases ERManI (MAN1B1) and initiates endoplasmic reticulum (ER)-associated protein degradation (ERAD) pathway degradation process.
The properties of ERMAN1 enable rapid selection of endoplasmic reticulum-associated degradation substrates in the endoplasmic reticulum.ERMAN1 digests mono-, di- and tri (zeige VANGL2 Antikörper)-glucosylated N-glycans.
we linked mutations in MAN1B1 to a Golgi glycosylation disorder. Additionally, our results support the recent findings on MAN1B1 localization
MAN1B1 deficiency appeared to be a frequent cause in our cohort of patients with unsolved congenital disorder of glycosylation type II.
A novel post-transcriptional regulatory mechanism for ERManI via miR (zeige MLXIP Antikörper)-125b and this molecule contributes to the regulation of carcinogenesis in hepatocellular carcinoma.
ERManI and gamma-COP (zeige COPg1 Antikörper) contribute to a golgi-based quality control module that facilitates the retrieval of captured ERAD substrates back to the endoplasmic reticulum.
A homozygous nonsense mutation in MAN1B1 segregated with nonsyndromic autosomal-recessive intellectual disability and additional dysmorphic features.
ER processing alpha1,2-mannosidase (zeige MAN1A2 Antikörper) (ER ManI (zeige FAM168B Antikörper)) has a role in ER-associated degradation of misfolded proteins
Modification by endoplasmic reticulum mannosidase I (ERManI) contributes to the preferential selection of the misfolded AAT (zeige SERPINA1A Antikörper) monomer for proteasomal degradation.
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.
mannosidase, alpha, class 1B, member 1
, alpha 1,2-mannosidase
, ER alpha 1,2-mannosidase
, Man9GlcNAc2-specific processing alpha-mannosidase
, endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase
, endoplasmic reticulum mannosyl-oligosaccharide 1,2-alpha-mannosidase 1
, ER alpha-1,2-mannosidase
, ER mannosidase 1
, man9GlcNAc2-specific-processing alpha-mannosidase