Tectonin beta-propeller repeat-containing protein 2 (TECPR2)

Tectonin beta-propeller repeat-containing protein 2 (TECPR2) is a large, multi-domain scaffold protein characterized by WD repeat domains at the N-terminus and TECPR repeats with a functional LC3-interacting region at the C-terminus[1][4][8][10]. TECPR2 is essential for autophagy, facilitating autophagosome formation, and mediating their targeting and fusion with lysosomes through interactions with ATG8 family members and tethering complexes such as BLOC1 and HOPS[1][4][8]. It coordinates secretory pathway trafficking by connecting the endoplasmic reticulum, Golgi apparatus, and vesicle export machinery, ensuring efficient maturation of autophagosomes and lysosomal degradation[1][2]. Loss-of-function mutations cause neurodegenerative syndromes, primarily spastic paraplegia type 49 (SPG49), intellectual disability, and hereditary sensory/autonomic neuropathies due to impaired autophagy and neuronal waste clearance[3][4][5][8][10]. TECPR2 is not classified as a receptor, enzyme, or canonical drug target, but is significant for understanding cellular waste management and neurodegenerative disease mechanisms.

Other names

TECPR2KIAA0329SPG49WD repeat-containing protein KIAA0329/KIAA0297tectonin beta-propeller repeat-containing protein 2 isoform 1/2