GMPPB Rabbit pAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q9Y5P6 |
| Other Accession | Q9Y5P6 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 39834 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human GMPPB |
| Epitope Specificity | 111-210/360 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the transferase hexapeptide repeat family. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | GMPPB is a 360 amino acid protein that belongs to the transferase hexapeptide repeat family and is involved in protein modification pathways. Functioning as a GDP-mannose pyrophosphorylase, GMPPB enzymatically catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose and a free phosphate, a reaction that is involved in the production of N-linked oligosaccharides. Defects in the gene encoding GMPPB that cause errors in the glycosylation pathway may lead to congenital disorders of glycosylation (CDG). CDGs are multisystemic diseases that often involve both the central and peripheral nervous systems and are often characterized by endocrine and coagulation disorders. GMPPB is expressed as two isoforms due to alternative splicing events. |
| Gene ID | 29925 |
|---|---|
| Other Names | LGMDR19; MDDGA14; MDDGB14; MDDGC14; E430010H19; RGD1560458; GMPPB_HUMAN; GMPPB; GDP-mannose pyrophosphorylase B; GTP-mannose-1-phosphate guanylyltransferase beta; 2.7.7.13; GMPPB_MOUSE; mannose-1-phosphate guanyltransferase beta |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | GMPPB (HGNC:22932) |
|---|---|
| Function | 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). |
| Cellular Location | Cytoplasm. |
| Tissue Location | [Isoform 1]: Ubiquitously expressed, including in brain and skeletal muscle. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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