CHM Antibody

CAT: 0223-60-861Size: 400 µLDry Ice: NoHazardous: No
CAT#:0223-60-861Size:400 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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

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