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Home / Primary Antibodies / Polyclonal Antibodies

CHM Antibody / Choroideremia protein

SKU : BHA17109056

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NSJ Bioreagents

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Mfr.No.	RQ4174
Clonality	Polyclonal
Description	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.
Formulation	0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host	Rabbit
Immunogen Region	A recombinant human protein corresponding to amino acids A193-Q380 was used as the immunogen for the CHM antibody.
Isotype	IgG
Predicted Reactivity	Human
Reactivity	Human
Recombinant	No

Antigen	CHM
Uniprot	P24386

Buffer	Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format	Antigen affinity purified
Purification	Antigen affinity purified
Storage	After reconstitution, the CHM antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.

Applications	WB, ELISA
Dilution	Western Blot: 0.5-1ug/ml,Direct ELISA: 0.1-0.5ug/ml

Product Manual	Data Sheet

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SKU	Supplier No.	Size	Your price	Quantity
BHA17109056	RQ4174	100ug	$439		Add to Cart

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