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.
Applications:Suitable for use in Western Blot and ELISA.
Other applications not tested.
Recommended Dilutions:Optimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A and peptide affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish peroxidase (HRP).
Specificity：Recognizes human CHM.
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu
Immunogen：KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 292-320 in the central region of human CHM.
