Overview |
bs-5021R |
ACOX1 Polyclonal Antibody |
WB, ELISA |
Human, Mouse, Rat, Sheep, Pig, Rabbit |
Specifications |
Unconjugated |
Rabbit |
KLH conjugated synthetic peptide derived from human ACOX1 |
221-320/660 |
Polyclonal |
IgG |
1ug/ul |
Purified by Protein A. |
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
Target |
51 |
Cytoplasm |
ACOX1; ACOX1_HUMAN; AOX antibody Palmitoyl CoA oxidase; Palmitoyl-CoA oxidase; Peroxisomal acyl coenzyme A oxidase 1; Peroxisomal acyl-coenzyme A oxidase 1; SCOX; Straight chain acyl CoA oxidase; Straight-chain acyl-CoA oxidase. |
Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD); also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. |
Application Dilution |
WB |
1:300-5000 |
ELISA |
1:500-1000 |