CHML polyclonal, anti-human, mouse, rat
€305.00
In stock
SKU
BS5889
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.
Alternative Name:
Rab proteins geranylgeranyltransferase component A 2, Choroideremia-like protein, Rab escort protein 2, REP-2, REP2
Application Dilution: WB: 1:500~1:1000, IHC: 1:50~1:200
Specificity: CHML polyclonal antibody detects endogenous levels of CHML protein.
Immunogen:
Synthetic peptide, corresponding to amino acids 170-215 of Human CHML.
MW: ~ 74 kDa
Swis Prot.: P26374
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).
Format:
1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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.
Alternative Name:
Rab proteins geranylgeranyltransferase component A 2, Choroideremia-like protein, Rab escort protein 2, REP-2, REP2
Application Dilution: WB: 1:500~1:1000, IHC: 1:50~1:200
Specificity: CHML polyclonal antibody detects endogenous levels of CHML protein.
Immunogen:
Synthetic peptide, corresponding to amino acids 170-215 of Human CHML.
MW: ~ 74 kDa
Swis Prot.: P26374
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).
Format:
1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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