Application
Western blot analysis in 293 cell line lysates (35ug/lane).| Product Name | CHM Antibody |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | Rab proteins geranylgeranyltransferase component A 1, Choroideremia protein, Rab escort protein 1, REP-1, TCD protein, CHM, REP1, TCD |
| Product description | This gene encodes component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Rab GTPases need to be geranylgeranyled on either one or two cysteine residues in their C-terminus to localize to the correct intracellular membrane. Mutations in this gene are a cause of choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. Alternative splicing results in multiple transcript variants encoding different isoforms.1) Perez-Cano, H.J., et al. Am. J. Med. Genet.149A (10), 2134-2140 (2009) : |
| Immunogen | This CHM antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 292-320 amino acids from the Central region of human CHM. |
| Clonality | Polyclonal |
|---|---|
| Isotype | Ig |
| Host Species | Rabbit |
| Tested Applications | WB |
| For WB starting dilution is: 1:1000: |
|
| Species Reactivity | Human |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Gene Symbol | CHM |
|---|---|
| Alternative Names | Rab proteins geranylgeranyltransferase component A 1 Choroideremia protein Rab escort protein 1 REP-1 TCD protein CHM REP1 TCD |
| Molecular Weight(MW) | 73 kDa |
Application
Western blot analysis in 293 cell line lysates (35ug/lane).| Application Notes | For WB starting dilution is: 1:1000: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures. |
| Storage Buffer | Supplied in PBS with 0.09% (W/V) sodium azide. |
