CHML polyclonal Antibody | BS5889

CHML polyclonal Antibody | BS5889 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse,Rat

Application: WB IHC

Application Range: WB: 1:500~1:1000 IHC: 1:50~1:200

Background: Two isoforms of the REP gene have been isolated, REP-1 and REP-2. The REP-1 gene, located on chromosome Xq21, is prone to a wide variety of mutations, including nonsense, frameshift and splice-site mutations and deletions. In patients with choroideraemia (CHM), mutations in the REP-1 gene result in progressive dystrophy of the choroid, retinal pigment epithelium and retina. CHM is an X-linked hereditary eye disease that leads to blindness later in life. REP-2 is able to bind to several Rab proteins with the same affinity as REP-1 and may act a substitute for REP-1 to prevent widespread tissue abnormalities in patients with CHM.

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: CHML polyclonal Antibody detects endogenous levels of CHML protein.

Molecular Weight: ~ 74 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: Rab proteins geranylgeranyltransferase component A 2; Choroideremia-like protein; Rab escort protein 2; REP-2; REP2

Immunogen: Synthetic peptide, corresponding to amino acids 170-215 of Human CHML.

Conjugate: N/A

Modification: N/A

Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Pathway: N/A