Technical Data

033849-HRP
Clone Type
Polyclonal
Host
Rabbit
Source
Human
Conjugate
HRP
Isotype
IgG
Grade
Affinity Purified
Applications
E WB
Crossreactivity
Hu
Accession #
NP_000381.1; P2438
Gene ID
1121
Gene #
CHM
Shipping Temp
Blue Ice
Storage Temp
-20°C
Notes
Preservative Free
Rabbit Anti-CHM, ID (CHM, REP1, TCD, Rab proteins geranylgeranyltransferase component A 1, Choroideraemia protein, Rab escort protein 1, TCD protein) (Azide free) (HRP)
CHM, REP1, TCD, Rab proteins geranylgeranyltransferase component A 1, Choroideraemia protein, Rab escort protein 1, TCD protein

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.
Immunogen
KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 292-320 in the central region of human CHM.
Form
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish peroxidase (HRP).
Purity
Purified by Protein A and peptide affinity chromatography.
Specificity
Recognizes human CHM.

Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.

References
1. Perez-Cano, H.J., et al. Am. J. Med. Genet. A 149A (10), 2134-2140 (2009) 2. Renner, A.B., et al. Arch. Ophthalmol. 127(7):907-912(2009) 3. Sergeev, Y.V., et al. Mutat. Res. 665 (1-2), 44-50 (2009) 4. MacDonald, I.M., et al. Surv Ophthalmol 54(3):401-407(2009) 5. Strunnikova, N.V., et al. PLoS ONE 4 (12), E8402 (2009)
USBio References
No references available
United States Biological | 4 Technology Way | Salem, MA 01970
Phone 800-520-3011 | Fax 978-594-8052 | Website www.usbio.net