The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 35 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
FunctionMediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis.
Tissue specificityLiver, fibroblasts, and placenta.
Involvement in diseaseDefects in SCP2 are a cause of leukoencephalopathy with dystonia and motor neuropathy (LDMN) [MIM:613724]; also known as sterol carrier protein 2 deficiency. LDMN is a syndrome characterized by leukoencephalopathy, dystonic head tremor, spasmodic torticollis and reduced tendon reflexes in lower extremities. Additional features include hyposmia, pathologic saccadic eye movements, a slight hypoacusis, accumulation of branched-chain pristanic acid in plasma, and the presence of abnormal bile alcohol glucuronides in urine.
Sequence similaritiesIn the N-terminal section; belongs to the thiolase family. Contains 1 SCP2 domain.
Cellular localizationMitochondrion; Cytoplasm. Mitochondrion. Cytoplasmic in the liver and also associated with mitochondria especially in steroidogenic tissues and Peroxisome. Interaction with PEX5 is essential for peroxisomal import.