Optineurin (OPTN) is a multifunctional adaptor protein that mainly involved in autophagy. While it was defined as an autophagy receptor and inducer, the extensive and important role in autophagy is hardly mentioned. What’s more, there are some critical disorders are closely related to OPTN, such as Glaucoma, Amyotrophic lateral sclerosis, Paget’s disease of bone, Crohn’s disease, cancer and diabetic nephropathy. This review will depict the OPTN involved autophagic process, from the recognition of cargo to the degradation in the autolysosome in molecular terms, and in particular highlight the process that compromised in disease related to OPTN. And we looking forward to strategies to modulate OPTN specifically with no toxic effects so that establish an efficient therapy for autophagy defective diseases.