REEP1 Rabbit pAb
REEP1 Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application
| WB |
|---|---|
| Primary Accession | Q9H902 |
| Reactivity | Mouse |
| Predicted | Rat, Pig, Dog, Human, Rabbit, Chicken, Horse, Sheep |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 22255 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human REEP1 |
| Epitope Specificity | 53-150/201 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Membrane. Mitochondrion membrane; Multi-passmembrane protein. Endoplasmic reticulum. |
| DISEASE | Defects in REEP1 are the cause of spastic paraplegia autosomal dominant type 31 (SPG31) [MIM:610250]. Spastic paraplegia is a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | Transport of G protein-coupled receptors (GPCRs) to the cell surface membrane is critical for receptor-ligand recognition. Mammalian GPCR odorant receptors (ORs), when heterologously expressed in cells, are poorly expressed on the cell surface. REEP1 (receptor expression-enhancing protein 1), is a 201 amino acid multi-pass mitochondrion membrane protein that belongs to the DP1 family. REEP1 interacts with odorant receptor proteins and may enhance the cell surface expression of odorant receptors. Mutations in the REEP1 gene are the third most common cause of hereditary spastic paraplegia (HSP) after spastin and atlastin gene mutations. Mutations in the REEP1 gene also cause spastic paraplegia autosomal dominant type 31, a neurodegenerative disorder. The REEP1 gene is conserved in chimpanzee, dog, cow, mouse, rat, chicken, zebrafish, A.thaliana and rice, and maps to human chromosome 2p11.2. |
| Gene ID | 65055 |
|---|---|
| Other Names | Receptor expression-enhancing protein 1, Spastic paraplegia 31 protein, REEP1, C2orf23, SPG31 {ECO:0000303|PubMed:23969831} |
| Dilution | WB=1:500-2000 |
| 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 | REEP1 |
|---|---|
| Synonyms | C2orf23, SPG31 {ECO:0000303|PubMed:23969 |
| Function | Required for endoplasmic reticulum (ER) network formation, shaping and remodeling; it links ER tubules to the cytoskeleton. May also enhance the cell surface expression of odorant receptors (PubMed:20200447). May play a role in long-term axonal maintenance (PubMed:24478229). |
| Cellular Location | Membrane. Mitochondrion membrane; Multi-pass membrane protein. Endoplasmic reticulum Note=Localizes to endoplasmic reticulum tubular network |
| Tissue Location | Expressed in circumvallate papillae and testis. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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