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GeneBio Systems

Anti-CHM Antibody Picoband®

Anti-CHM Antibody Picoband®

SKU:A00814-2

Regular price €506,95 EUR
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Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human

Applications: Flow Cytometry,IF,IHC,ICC,WB

Application Details: Western blot, 0.1-0.5μg/ml
Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml
Immunocytochemistry/Immunofluorescence, 5μg/ml
Flow Cytometry (Fixed), 1-3μg/1x106 cells

Gene Name: CHM

Specificity: No cross reactivity with other proteins.

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. It is mapped to Xq21.2. 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.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human CHM, which shares 88.9% amino acid (aa) sequence identity with both mouse and rat CHM.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Entrez Gene: CHM choroideremia (Rab escort protein 1). 2. Molloy CM, van de Pol TJ, Brohet RM, Ropers HH, Cremers FP (May 1992). Three RFLPs for pZ11 (DXS540) in the choroideremia gene at Xq21.2. Nucleic Acids Res. 20 (6): 1434.

Uniprot ID: P24386

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate: This is the unconjugated antibody. It is also available with 10 conjugation options, including Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, and Fluoro647. A carrier-free (BSA-free) version is also available. Additional charges may apply for some options. Please contact us for a quote

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 100 kDa

Calculated Molecular Weight: 53384 MW

Gene ID: 1121

Protein Name: Rab proteins geranylgeranyltransferase component A 1

Gene Full Name: CHM Rab escort protein

Synonyms: CHM; Choroideraemia protein; GGTA; REP1; TCD

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