In sum, while the pathophysiological consequences of TMEM175 dysfunction due to specific point mutations are becoming increasingly evident, for example, more and more TMEM175 mutation‐associated PD cases are being discovered, some uncertainty remains, as outlined above as to whether too much or too little TMEM175 activity or both may have negative implications, in particular for the development or for the progression of neurodegenerative diseases such as PD. Here, TMEM175 is linked to Parkinson disease.