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>   首页   >   产品   >   一抗   >   信号转导   >   Anti-CHM antibody   

Anti-CHM antibody

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, E
Primary Accession A00814-1
Host Rabbit
Reactivity Human, Mouse, Rat
Clonality Polyclonal
Format Lyophilized
Description Rabbit IgG polyclonal antibody for CHM detection. Tested with WB, Direct ELISA in Human;Mouse;Rat.
Reconstitution Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
Additional Information
Application Details Western blot, 0.1-0.5 µg/ml

Direct ELISA, 0.1-0.5 µg/ml
Subcellular Localization Cytoplasm, cytosol.
Contents Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
Immunogen E. coli-derived human CHM recombinant protein (Position: A193-Q380).
Cross Reactivity No cross reactivity with other proteins.
Storage At -20˚C; for one year. After r˚Constitution, at 4˚C; for one month. It˚Can also be aliquotted and stored frozen at -20˚C; for a longer time. Avoid repeated freezing and thawing.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Research Areas

BACKGROUND

Rab escort protein 1 (REP1) also known as rab proteins geranylgeranyltransferase component A 1 is an enzyme that in humans is encoded by the CHM gene. 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. Alternatively spliced transcript variants have been found for this gene.

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