RBM28 Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q9NW13 |
| Other Accession | Q9NW13 |
| Predicted | Human, Mouse, Rabbit, Dog, Horse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 85738 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human RBM28 |
| Epitope Specificity | 601-700/759 |
| 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 4 RRM (RNA recognition motif) domains. |
| DISEASE | Defects in RBM28 are the cause of alopecia neurologic defects and endocrinopathy syndrome (ANES) [MIM:612079]. Affected individuals have hair loss of variable severity, ranging from complete alopecia to near-normal scalp hair with absence of body hair. All have moderate to severe mental retardation, progressive motor deterioration and central hypogonadotropic hypogonadism with delayed or absent puberty and central adrenal insufficiency. Additional features included short stature, microcephaly, gynecomastia, pigmentary anomalies, hypodontia, kyphoscoliosis, ulnar deviation of the hands, and loss of subcutaneous fat. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | The protein encoded by this gene is a specific nucleolar component of the spliceosomal small nuclear ribonucleoprotein (snRNP)complexes . It specifically associates with U1, U2, U4, U5, and U6 small nuclear RNAs (snRNAs), possibly coordinating their transition through the nucleolus. Mutation in this gene causes alopecia, progressive neurological defects, and endocrinopathy (ANE syndrome), a pleiotropic and clinically heterogeneous disorder. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009] |
| Gene ID | 55131 |
|---|---|
| Other Names | ANES; NOP4; RBM28_HUMAN; RBM28; RNA-binding motif protein 28; RNA binding motif protein 28 |
| 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 | RBM28 |
|---|---|
| Function | Nucleolar component of the spliceosomal ribonucleoprotein complexes. |
| Cellular Location | Nucleus, nucleolus {ECO:0000269|PubMed:12429849, ECO:0000269|PubMed:17081119, ECO:0000269|Ref.7} |
| Tissue Location | Ubiquitously expressed. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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