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KBTBD13 Rabbit pAb
- 产品详情
- 实验流程
Application 
| IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | C9JR72 |
| Other Accession | C9JR72 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 49485 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human KBTBD13 |
| Epitope Specificity | 381-458/458 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Contains 1 BTB (POZ) domain. Contains 5 Kelch repeats. |
| DISEASE | Defects in KBTBD13 are the cause of nemaline myopathy type 6 (NEM6) [MIM:609273]. A form of nemaline myopathy characterized by childhood onset of slowly progressive proximal muscle weakness, exercise intolerance, and slow movements with stiff muscles. Patients are unable to run or correct themselves from falling over. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | The gene belongs to a family of genes encoding proteins containing a BTB domain and several kelch repeats. The BTB domain functions as a protein-protein interaction module, which includes an ability to self-associate or to interact with non-BTB domain-containing proteins. The kelch motif typically occurs in groups of five to seven repeats, and has been found in proteins with diverse functions. Known functions of these family members include transcription regulation, ion channel tetramerization and gating, protein ubiquitination or degradation, and cytoskeleton regulation. The exact function of this family member has yet to be determined. [provided by RefSeq, Jun 2010] |
| Gene ID | 390594 |
|---|---|
| Other Names | HCG1645727; NEM6; KBTBD_HUMAN; KBTBD13; kelch repeat and BTB domain containing 13; kelch repeat and BTB (POZ) domain containing 13; nemaline myopathy type 6 |
| Dilution | 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 | KBTBD13 |
|---|---|
| Function | Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex. |
| Cellular Location | Cytoplasm |
| Tissue Location | Expressed in skeletal muscle. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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