JavaScript is disabled in your browser. Please enable JavaScript to view this website.
AB154061

Anti-GMPPB antibody

Be the first to review this product! Submit a review

|

(1 Publication)

Rabbit Polyclonal GMPPB antibody. Suitable for WB and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human GMPPB aa 1-250.

View Alternative Names

Mannose-1-phosphate guanylyltransferase catalytic subunit beta, GDP-mannose pyrophosphorylase B, GTP-mannose-1-phosphate guanylyltransferase beta, GMPPB

1 Images
Western blot - Anti-GMPPB antibody (AB154061)
  • WB

Unknown

Western blot - Anti-GMPPB antibody (AB154061)

10% SDS PAGE

All lanes:

Western blot - Anti-GMPPB antibody (ab154061) at 1/1000 dilution

All lanes:

IMR32 whole cell lysate at 30 µg

Predicted band size: 40 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

WB

applications

Immunogen

Recombinant Fragment Protein within Human GMPPB aa 1-250. The exact immunogen used to generate this antibody is proprietary information.

Q9Y5P6

style
left-column

Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/500 - 1/3000", "WB-species-notes": "<p></p>" }, "Mouse": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "" }, "Rat": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "" } } }
style
left-column
style
left-column

Properties and storage information

Form
Liquid
Purification technique
Affinity purification Immunogen
Storage buffer
pH: 7 Preservative: 0.01% Thimerosal (merthiolate) Constituents: PBS, 20% Glycerol (glycerin, glycerine), 1% BSA
Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
style
left-column

Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

GMPPB also known as GDP-mannose pyrophosphorylase B is an enzyme that catalyzes the conversion of mannose-1-phosphate and GTP into GDP-mannose. This enzyme has a molecular mass of approximately 38 kDa. GMPPB is expressed in various tissues throughout the body with significant levels in skeletal muscle and the brain. It is an essential enzyme for the proper synthesis of nucleotide sugars which play an important role in glycosylation processes.
Biological function summary

The activity of GMPPB influences the assembly and stability of glycoproteins by providing the necessary substrates for glycosylation. Glycosylation is significant for protein folding stability and cell-cell interactions. GMPPB does not function alone; it is involved in synergy with other enzymes in the GDP-mannose biosynthesis pathway. This pathway is important for the formation of glycosylated proteins and lipids affecting numerous cellular processes and signaling pathways.

Pathways

GMPPB is actively involved in the nucleotide sugar metabolism and protein glycosylation pathways. It collaborates with other enzymes like ALG1 and ALG2 which further facilitate the glycosylation processes in the endoplasmic reticulum. These pathways are integral for maintaining cellular communication and structural integrity ultimately impacting cellular homeostasis and function.

GMPPB mutations have been linked to congenital disorders of glycosylation and muscular dystrophies such as muscular dystrophy-dystroglycanopathy (MDDG). These conditions involve defective glycosylation leading to improper muscle and neural development and function. Abnormalities in proteins like dystrophin are associated with GMPPB mutations underlining their involvement in these genetic disorders. Understanding GMPPB's role provides insights into potential therapeutic targets for addressing these conditions.
style
left-column
style
left-column

Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
style
left-column

Target data

Catalytic subunit of the GMPPA-GMPPB mannose-1-phosphate guanylyltransferase complex (PubMed : 33986552). Catalyzes the formation of GDP-mannose, an essential precursor of glycan moieties of glycoproteins and glycolipids (PubMed : 33986552). Can catalyze the reverse reaction in vitro (PubMed : 33986552). Together with GMPPA regulates GDP-alpha-D-mannose levels (PubMed : 33986552).
See full target information GMPPB
style
left-column

Publications (1)

Recent publications for all applications. Explore the full list and refine your search

Brain : a journal of neurology 138:2493-504 PubMed26133662

2015

Mutations in GMPPB cause congenital myasthenic syndrome and bridge myasthenic disorders with dystroglycanopathies.

Applications

Unspecified application

Species

Unspecified reactive species

Katsiaryna Belaya,Pedro M Rodríguez Cruz,Wei Wei Liu,Susan Maxwell,Simon McGowan,Maria E Farrugia,Richard Petty,Timothy J Walls,Maryam Sedghi,Keivan Basiri,Wyatt W Yue,Anna Sarkozy,Marta Bertoli,Matthew Pitt,Robin Kennett,Andrew Schaefer,Kate Bushby,Matt Parton,Hanns Lochmüller,Jacqueline Palace,Francesco Muntoni,David Beeson
View all publications
style
left-column
style
left-column
style
right-column

Complementary Products

Secondary Antibodies

AB150077

Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488)

Immunocytochemistry/ Immunofluorescence - Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (AB150077)

  • 5
  • 20
Primary Antibodies

AB182981

Anti-CD31 antibody [EPR17259]

BOND RX™ Validated|RabMAb|Recombinant|Lab Essentials|20ul selling size|Advanced Validation

Multiplex immunohistochemistry - Anti-CD31 antibody [EPR17259] (AB182981)

  • 5
  • 22
Primary Antibodies

AB52922

Anti-HLA A antibody [EP1395Y]

RabMAb|Recombinant|KO Validated

Immunoprecipitation - Anti-HLA A antibody [EP1395Y] (AB52922)

  • 4
  • 15
Primary Antibodies

AB109496

Anti-HEC1/HEC antibody [EPR5342]

RabMAb|Recombinant

Western blot - Anti-HEC1/HEC antibody [EPR5342] (AB109496)

  • 0
  • 0

Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.
For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

For licensing inquiries, please contact partnerships@abcam.com