STBD1

STBD1, or starch binding domain-containing protein 1 (alias genethonin-1), is a cytosolic protein with a C-terminal starch-binding domain (SBD). It binds glycogen particles and colocalizes with lysosomes, mediating glycogen trafficking to lysosomes for degradation—critical for maintaining cellular glycogen homeostasis.
Dysregulation of STBD1 is linked to glycogen storage disorders (GSDs), especially those involving defective lysosomal glycogen breakdown. No approved drugs target STBD1 directly yet, but preclinical research explores modulating its glycogen-binding or lysosomal interactions to alleviate glycogen accumulation in GSD models.