Abstract: Adenosine triphosphate（ATP） binding cassette transporters, known as ABC transporters, is a kind of active transporters which can be found in all kingdoms of life. It normally consists of four domains: two transmembrane domains (TMD) and two nucleotide-binding domains (NBD). NBDs hydrolyzes ATPs, and the produced energy can be used by TMDs to transport substrates across the membrane against the chemical potential. ABC transporters anticipate in a variety of physiological process, because almost all functional molecules can be recognized as substrates by ABC transporters. The human genome encodes 48 ABC transporters in total, and dysfunction of the transporters is directly related to some major diseases. This essay summarized the classification and features of human ABCs, and emphasized the latest research progress of structure studies of human ABC transporters, as well as their relations with human diseases.