> Antigen, Antibodies, ELISA, Western Blot > Primary Antibody > Polyclonal Antibodies > CHM Antibody (Center)Brand |
Leading Biology | Catalog Number |
AMM05747G |
Product Type |
Polyclonal Antibodies | Field of Research |
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Product Overview |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality CHM antibody (Center).
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Molecular Weight |
73476 Da
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Cellular Localization |
Antigen Cellular Localization:
Cytoplasm, cytosol
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Host |
Rabbit
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Species Reactivity |
Human
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Immunogen |
292-320 aa
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Target |
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.
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Isotype |
Rabbit Ig
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Symbol |
REP1, TCD
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GeneID |
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UniProt ID |
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Function |
Substrate-binding subunit of the Rab geranylgeranyltransferase (GGTase) complex. Binds unprenylated Rab proteins and presents the substrate peptide to the catalytic component B composed of RABGGTA and RABGGTB, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Besides, a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation.
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Summary |
This gene encodes component A of the RAB geranylgeranyltransferase holoenzyme. In the dimeric holoenzyme, this subunitbinds unprenylated Rab GTPases and then presents them to thecatalytic Rab GGTase subunit for the geranylgeranyl transferreaction. Rab GTPases need to be geranylgeranyled on either one ortwo cysteine residues in their C-terminus to localize to thecorrect intracellular membrane. Mutations in this gene are a causeof choroideremia; also known as tapetochoroidal dystrophy (TCD).This X-linked disease is characterized by progressive dystrophy ofthe choroid, retinal pigment epithelium and retina. Alternativesplicing results in multiple transcript variants encoding differentisoforms.
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Form |
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
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Storage & Stability |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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Applications |
WB, E
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Dilution |
WB~~1:1000
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Synonyms |
Rab proteins geranylgeranyltransferase component A 1, Choroideremia protein, Rab escort protein 1, REP-1, TCD protein, CHM, REP1, TCD
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Images |
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Specification |
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Quantity |
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Leading Biology Inc.
2600 Hilltop DR, Building G, B Suite C138
Richmond, CA, 94806
Tel: 1-661-524(LBI)-0262
Email: info@leadingbiology.com
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