Sign in or Register   Sign in or Register
  |  

Mouse Anti-POMK Recombinant Antibody (1B1) (CBMAB-P2391-YC)

Provided herein is a Mouse monoclonal antibody against Human Protein-O-Mannose Kinase. The antibody can be used for immunoassay techniques, such as IHC-P, WB.
See all POMK antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1B1
Antibody Isotype
IgG2a
Application
IHC-P, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
PBS, pH 7.3, 1% BSA, 50% glycerol, 0.02% sodium azide
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 141-350

Target

Full Name
protein-O-mannose kinase
Introduction
POMK is a protein that may be involved in the presentation of the laminin-binding O-linked carbohydrate chain of alpha-dystroglycan (a-DG), which forms transmembrane linkages between the extracellular matrix and the exoskeleton. Some pathogens use this O-linked carbohydrate unit for host entry. Loss of function compound heterozygous mutations in this gene were found in a human patient affected by the Walker-Warburg syndrome (WWS) phenotype. Mice lacking this gene contain misplaced neurons (heterotopia) in some regions of the brain, possibly from defects in neuronal migration.
Entrez Gene ID
84197
UniProt ID
Q9H5K3
Alternative Names
MDDGA12; MDDGC12; SGK196
Function
Protein O-mannose kinase that specifically mediates phosphorylation at the 6-position of an O-mannose of the trisaccharide (N-acetylgalactosamine (GalNAc)-beta-1,3-N-acetylglucosamine (GlcNAc)-beta-1,4-mannose) to generate phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-1,3-N-acetylglucosamine-beta-1,4-(phosphate-6-)mannose). Phosphorylated O-mannosyl trisaccharide is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Only shows kinase activity when the GalNAc-beta-3-GlcNAc-beta-terminus is linked to the 4-position of O-mannose, suggesting that this disaccharide serves as the substrate recognition motif.
Biological Process
Brain developmentManual Assertion Based On ExperimentTAS:UniProtKB
Carbohydrate phosphorylationManual Assertion Based On ExperimentIDA:UniProtKB
Learning or memoryIEA:Ensembl
Neuromuscular processIEA:Ensembl
Protein O-linked glycosylationManual Assertion Based On ExperimentIDA:UniProtKB
Protein phosphorylationIEA:InterPro
Sensory perception of painIEA:Ensembl
Cellular Location
Endoplasmic reticulum membrane
Involvement in disease
Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A12 (MDDGA12):
An autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound intellectual disability, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye-brain disease.
Muscular dystrophy-dystroglycanopathy limb-girdle C12 (MDDGC12):
An autosomal recessive limb-girdle congenital muscular dystrophy, characterized by muscle weakness and delayed motor development in association with cognitive impairment.
Topology
Cytoplasmic: 1-20
Helical: 21-43
Lumenal: 44-350
Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-POMK Recombinant Antibody (1B1)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare