Description
87047-1-RR targets ABCD3 in WB, IF/ICC, ELISA applications and shows reactivity with human, mouse, rat samples.
Tested Applications
Positive WB detected in: mouse liver tissue, PC-3 cells, PC-12 cells
Positive IF/ICC detected in: PC-3 cells
Recommended dilution
Western Blot (WB): WB : 1:2000-1:10000
Immunofluorescence (IF)/ICC: IF/ICC : 1:250-1:1000
Background Information
ABCD3 belongs to the superfamily of ATP-binding cassette (ABC) transporters and is a peroxisomal membrane transporter involved in the transport of branched-chain fatty acids and C27 bile acids into the peroxisome. ABCD3 plays a role in the regulation of LCFAs and energy metabolism, namely in the degradation and biosynthesis of fatty acids via beta-oxidation (PMID: 24333844). Mutations in ABCD3 are associated with congenital bile acid synthesis defect 5 (CBAS5)(PMID: 25168382).