BS5889

antibody from Bioworld Technology, Inc
Targeting: CHML REP-2
Provider product page for BS5889
Western blot
Immunohistochemistry

Antibody data

Product number
BS5889 - Provider product page
Provider
Bioworld Technology, Inc
Product name
CHML polyclonal antibody
Antibody type
Polyclonal
Antigen
Synthetic peptide, corresponding to amino acids 170-215 of Human CHML.
Description
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.
Reactivity
Human, Mouse, Rat
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
CHML protein structure - BS5889 shown in red.