GLCNE Polyclonal Antibody, Biotin Conjugated
Applications
Reactivity
| Overview | |
| Catalog # | bs-9882R-Biotin-100uL |
| Product Name | GLCNE Polyclonal Antibody, Biotin Conjugated |
| Applications |
WB
IHC-P
|
| Reactivity | Human, Mouse, Rat |
| Specifications | |
| Conjugation | Biotin |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human GLCNE |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C for 12 months. |
| Target | |
| Gene ID | 10020 |
| Synonyms | IBM2; Uae1; Bunctional UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase; DMRV; ManAc kinase; N acylmannosamine kinase; NM; RP23-209M8.6; UDP GlcNAc 2 epimerase; UDP GlcNAc 2 epimerase/ManAc kinase; Uridine diphosphate N acetylglucosamine 2 epimerase; GLCNE_HUMAN. |
| Background | The bifunctional enzyme UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE/Mnk), or GLCNE, regulates and initiates biosynthesis of N-acetylneuraminic acid (NeuAc), a precursor of sialic acids. GLCNE is required for normal sialylation in hematopoietic cells. Sialylation is implicated in cell adhesion, signal transduction, tumorigenicity and metastatic behavior of malignant cells. It is upregulated after PKC-dependent phosphorylation and is most abundantly expressed in liver and placenta. It is also expressed, to a lesser extent, in heart, brain, lung, kidney, skeletal muscle and pancreas. Defects in GLCNE are the cause of sialuria, inclusion body myopathy 2 (IBM2) and Nonaka myopathy (NM) or distal myopathy with rimmed vacuoles (DMRV). Sialuria is an autosomal dominant disorder caused by a lack of feedback inhibition of GLCNE by CMP-NeuAc, resulting in overproduction of NeuAc. It is characterized by an accumulation of free sialic acid in the cytoplasm and large quantities of neuraminic acid in the urine. Both IBM2 and NM/DMRV are autosomal recessive neuromuscular disorders characterized by adult onset, distal and proximal muscle weakness (especially in the legs) and a typical muscle pathology including filamentous inclusions and rimmed vacuoles. |
| Application Dilution | |
| WB | 1:300-5000 |
| IHC-P | 1:200-400 |
Frequently asked questions
Which applications is GLCNE Polyclonal Antibody, Biotin Conjugated (bs-9882R-Biotin) validated for, and at what dilution?
bs-9882R-Biotin is validated for WB (1:300-5000); IHC-P (1:200-400). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
Which species does GLCNE Polyclonal Antibody, Biotin Conjugated (bs-9882R-Biotin) react with?
Tested reactivity: Human, Mouse, Rat.
How should bs-9882R-Biotin be stored?
Store at -20C for 12 months. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise GLCNE Polyclonal Antibody, Biotin Conjugated (bs-9882R-Biotin)?
KLH conjugated synthetic peptide derived from human GLCNE