CHM Antibody

• Background:

  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.

• NCBI Gene ID:

  1121

• Swiss Prot:

  P24386

• Accession Number:

  P24386

• Host:

  Rabbit

• Reactivity:

  Human

• 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

• Conjugation:

  Unconjugated

• Type:

  Primary Antibodies

• Field of Research:

  Neuroscience, Signal Transduction

• Purification:

  This antibody is purified through a protein A column, followed by peptide affinity purification.

• Concentration:

  Batch dependent

• Buffer:

  Supplied in PBS with 0.09% (W/V) sodium azide.

• Modification:

  None

• Shipping Conditions:

  Blue Ice

• Storage Conditions:

  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.

• Calculated Molecular Weight:

  73 kDa

• Fragment:

  Rabbit Ig

• Applications Notes:

  For WB starting dilution is: 1:1000

• Symbol:

  CHM

• NCBI Official Name:

  Rab proteins geranylgeranyltransferase component A 1

• NCBI Organism:

  Homo sapiens

• Background Reference 01:

  Perez-Cano, H.J., et al. Am. J. Med. Genet.149A (10), 2134-2140 (2009) :

• Background Reference 02:

  Renner, A.B., et al. Arch. Ophthalmol. 127 (7) :907-912 (2009)

• Background Reference 03:

  Sergeev, Y.V., et al. Mutat. Res. 665 (1-2), 44-50 (2009) :

• Background Reference 04:

  MacDonald, I.M., et al. Surv Ophthalmol 54 (3) :401-407 (2009)

• Other Product Names:

  Rab proteins geranylgeranyltransferase component A 1, Choroideremia protein, Rab escort protein 1, REP-1, TCD protein, CHM, REP1, TCD

• Tested Applications:

  WB

• Protein ID:

  21431807

• Physical Properties:

  Liquid